Neurovascular Pathology Flashcards
(788 cards)
1
Q
What is myopia?
A
Short-sightedness (close objects appear clear, distant objects are hazy).
2
Q
What causes myopia?
A
An eyeball that is too long. Cornea and lens make image form in front of retina.
3
Q
What symptoms are associated with myopia?
A
Headaches, unable to see in distance, divergent squint in children, toddlers lose interest in sports/people, more interested in books/pictures.
4
Q
How is myopia treated?
A
Biconcave lenses (glasses/contact lenses), laser eye surgery.
5
Q
What is hyperopia?
A
Long-sightedness (close objects hazy, far off images clear).
6
Q
What causes hyperopia?
A
Mostly due to eyeball being too small or cornea/lens being too fat. Image formed behind retina. Person uses accommodative power to make lens thicker to allow image to be formed on retina, so when seeing close up, they use more and more power until they cannot see properly.
7
Q
What symptoms are associated with hyperopia?
A
Eyestrain after working/reading on computer, convergent squint in children (needs immediate correct to prevent vision loss/lazy eye).
8
Q
What is astigmatism?
A
Image is always hazy, regardless of distance of object.
9
Q
What causes astigmatism?
A
Surface of eye has different curvatures in different meridians, so bending of light along one axis will never be the same as that of another axis. Cornea is rugby ball shaped.
10
Q
How is astigmatism treated?
A
Cylindrical glasses (only curved in once axis), toric lenses (weighted to be put on correctly), laser eye surgery.
11
Q
How is hyperopia corrected?
A
Biconvex lenses (contact lenses, or glasses), laser eye surgery.
12
Q
What happens in presbyopia?
A
With age, lens becomes less immobile/elastic. Ciliary muscle contraction is unable to change lens shape. Seeing near objects becomes increasingly difficult.
13
Q
When does presbyopia tend to occur?
A
Usually starts in 50s.
14
Q
What symptoms are associated with presbyopia?
A
Eyestrain, difficulty reading small print, headaches.
15
Q
How is presbyopia treated?
A
Biconcave lenses (reading glasses).
16
Q
What are the signs and symptoms of vitamin A deficiency?
A
(Night) blindness, Bitot’s spots (silvery triangular spot in conjunctiva), corneal ulceration and corneal melting.
17
Q
What complications can result from vitamin A deficiency?
A
In serious cases - opacification of cornea.
18
Q
What can cause vitamin A deficiency?
A
Any condition affecting vitamin A absorption, e.g. malnutrition, malabsorption syndromes, e.g. coeliac disease or sprue.
19
Q
What do Bitot’s spots result from?
A
Build up of keratin.
20
Q
What will damage to the right optic nerve result in?
A
Blindness in right eye.
21
Q
What will damage to the middle of optic chiasma cause?
A
Bilateral temporal hemianopia.
22
Q
What will damage to the right optic tract cause?
A
Left homonymous hemianopia.
23
Q
What will damage to the right optic radiation?
A
Left homonymous hemianopia.
24
Q
What is strabismus?
A
Squint - misalignment of the eyes.
25
What are the two types of strabismus?
Estropia (convergent squint), exotropia (divergent squint).
26
What are the functional consequences of strabismus?
Amblyopia, diplopia.
27
In what kind of patients does amblyopia happen more commonly?
If one eye if hyperopic, myopic or astigmatic.
28
What causes amblyopia?
Aka. - lazy eye
| Brain surpasses image of one eye leading to poor vision in that eye without any pathology.
29
How is strabismus corrected?
Glasses.
30
How is amblyopia corrected?
Corrected in early years by using patches to stimulate lazy eye.
31
What is anioscoria?
When the pupils are different sizes - can be harmless or sign of underlying medical problems.
32
What can anioscoria indicate?
Horner's syndrome.
33
What are reasons for the pupils reacting abnormally to light?
Abnormalities of the afferent limb/centre/efferent limb of cranial nerve III
IIIn palsy due to medical cause
Diseases of the retina (detachment, degenerations, dystrophies)
Diseases of the optic nerve, e.g. optic neuritis in MS.
34
What is important to remember in patients with IIIn palsy and absent pupillary reflex with diabetes?
Diabetes tends not to cause damage to the parasympathetic fibres of cranial nerve III, so if you see patient with IIIn palsy and absent pupillary reflex suspect a cerebral artery aneurysm (medical emergency).
35
What is the cause of Horner's syndrome?
Occurs due to disruption of sympathetic supply to the eye, e.g. due to Pancoast tumour or stroke.
36
What are the signs associated with Horner's syndrome?
Anisocoria (miosis (excessive constriction of eye), hemifacial anhydrous (loss of sweating on affected side), enophthalmos, partial ptosis.
37
How do you investigate Horner's syndrome?
MRI, CT, Xray.
38
How do you treat Horner's syndrome?
Treat underlying medical condition.
39
What causes a blowout fracture?
Blunt force hitting the eye is transmitted to the orbit (orbital rim strong but orbit weak, structures can herniate into maxillary sinus).
40
What are the signs of a blowout fracture?
Infraorbital nerve sensation over infraorbital foramen (where it comes out) reduced.
41
What symptoms are associated with a blow out fracture?
Diplopia, inability to elevate one/both eyes.
42
How are blow out fractures managed?
Some cases small and heal on their own, others require surgery to put a wire mesh into the floor of the orbit.
43
In what kind of diseases do you get orbital fat hypertrophies?
Thyroid disease.
44
What are the normal cornea-scleral junctions (limbus)?
Normal upper eyelid 1-2mm below cornea-scleral junction and for lower eyelid at level of corneascleral junction.
45
What will you see on fundoscopy if infection spreads into the eyes and sinuses? How can the infection spread to the sinuses? What can this result in?
Swollen and tortuous veins, swollen and engorged optic disc (venous drainage from orbit compromised, and unless dealt with it can spread through emissary veins to the cavernous sinus leading to cavernous sinus thrombosis and cause compression/paralysis of movement).
46
What symptoms will you experience if infection spreads into the eyes and sinuses?
Red, painful eyes, no eye movement, can lead to total loss of vision.
47
What is a coloboma? When are they present from?
Hole in one of the structures of the eye, e.g. iris coloboma, retinal coloboma, optic disc coloboma). Present from birth or develop in first few months. Can be uni/bilateral.
48
What causes a coloboma?
Failure of fissure fusion.
49
What symptoms can people with coloboma experience?
May be asymptomatic or completely blind (if large part of retina/optic disc missing).
50
What can blunt trauma to the eye result in?
Peripheral retinal tear, vitreous gel becomes liquified and can push through the retinal tear and detach it.
51
What is conjunctivitis?
Self-limiting bacteria/viral infection leading to enlargement of blood vessels in conjunctiva, sometimes causing the eyelids to stick together.
52
What symptoms will someone with conjunctivitis experience?
Red, watery eyes, discharge (pus), no loss of vision (if doesn't spread to cornea).
53
What are the possible complications of conjunctivitis?
Blurry vision indicative of infection spread to cornea (adenovirus can do this).
54
How is conjunctivitis treated?
Antibiotic eye drops.
55
What is ptosis?
Drooping of the upper eyelid.
56
What can cause ptosis?
Levator palpebrae superiors paralysis (IIIn dystrophy or paralysis).
57
What are the signs of ptosis?
Inability to open the eyelid.
58
What are the two types of stye/hordeolum?
External - due to infection of sebaceous glands of the eyelids
Internal - due to infection of Meibomian glands (responsible for creating oil layer of tear film)
59
What is a stye/hordeolum?
Usually an abscess filled with pus caused by staphylococcus infection.
60
What symptoms are associated with styes?
Painful lump on inside or outside of eyelid. Vision should be affected. Eyelid or eye may be red/watery.
61
How can you treat styes?
Warm compress, eyelid hygiene, analgesia, may need surgical incision and curettage.
62
What are corneal ulcers? Which layer is important that you don't injure? What can cause corneal ulcers?
Inflammation of the cornea (epithelium lost, storm exposed if damage passes Bowman's layer), cornea exposed to outer environment, e.g. bacteria, viruses, fungi.
Non-infective causes include trauma, degeneration or dystrophy.
63
What symptoms are associated with corneal ulcers?
Red eye, pain.
64
What complications can result due to corneal ulcers?
Loss of vision.
65
How are corneal ulcers managed?
Aggressive Rx to prevent scarring, corneal transplant/keroplasty.
66
What are non-inflammatory corneal dystrophies?
A group of diseases affecting the cornea, which are bilateral, opacifying, non-inflammatory. These diseases will start in one layer and spread to others.
These are mostly genetically determined.
67
What causes the non-inflammatory corneal dystrophies?
Sometimes due to accumulation of lipids.
68
What are cataracts?
Clouding of the lens in the eye leading to decreased vision.
69
How do cataracts develop?
Develop as older embryological fibres of eye nerve shed and become compacted in the middle. There is no blood supply to the lens which depends entirely on diffusion for nutrition. Opacities can be quite immature (usually in cortex of lens (deeper part of nucleus is lens (older cells)).
70
What can cause cataracts?
Use of steroid eye drops, tumours, paging. Nuclear sclerosis is a type of cataract, UV rays.
71
What are sutural and zonular cataracts?
Types of childhood cataract due to opacification of certain zones of the lens in utero (due to mother malnutrition/German Measles contraction).
72
What are the risk factors for cataracts?
Diabetes, smoking.
73
What symptoms are associated with cataracts?
Night blindness (when pupil dilates if immature cataract), can use sympathetic agent to stimulate pupils, light sensitivity.
74
How are cataracts treated?
Day case surgery (small incision made, lens capsule opened, lens removed by emulsification, plastic lens is placed in capsular bag). Lens known posterior chamber intraocular lens (PCIOL).
75
What are the two types of glaucoma?
Open angle glaucoma or angle closure glaucoma.
76
What happens in a primary open angle glaucoma?
Angle between the cornea and iris is open. Drainage system slowly gets clogged over time leading to an increase in intralocular pressure. Pressure transferred to outer rim of nerve leading to peripheral vision loss. But as it increases leads to optic nerve damage, leading to loss in central vision loss as well.
77
Where is primary open angle glaucoma normally picked up?
Routine eye exams.
78
What will you see on ophthalmoscopy in primary open angle glaucoma?
Unhealthy, pale, cupped optic disc.
79
What are the triad of signs for the diagnosis of primary open angle glaucoma?
Raised IOP, visual field defects, optic disc changes on ophthalmoscopy.
80
What symptoms will someone with open angle glaucoma experience?
Can be asymptomatic for a long time, slow onset of symptoms, altered visual field and can result in total blindness.
81
How is primary open angle glaucoma treated?
Eye drops to decrease IOP - prostaglandin analogues, beta-blockers, carbonic anhydrase inhibitors), laser trabeculoplasty, trabeculectomy surgery (take off sclera and trabecular meshwork).
82
What happens in an angle closure glaucoma?
Angle between iris and cornea too small - passage for AH too narrow, leads to lens being pushed against the iris. Drainage system blocked and causes rapid pressure build up in eye. AH unable to reach trabecular meshwork, more formed and build up causes iris to ballon and close epithelium further.
83
What are the risk factors for angle closure glaucoma?
Tends to happen in predisposed eyes (long-sighted person, in dusk - blocks off angle).
84
What signs and symptoms are associated with angle closure glaucoma?
Signs - red eye, fixed dilated pupil.
Symptoms - abrupt onset of eye pain and redness, blurry vision, headaches, nausea, opacities as IOP leads to fluid leaking into cornea.
85
How is angle closure glaucoma managed?
Decrease IOP - IV infusion w/ or w/o therapy (CAH (acetazolamide), analgesia, anti-emetics, constrictor eye drops (pilocarpine), beta-blockers, e.g. timolol, steroid eye drops, e.g. dexamethasone, iridotomy (laser) both eyes to bypass block (make tiny hole to allow AH to come to angle and move about).
86
What is uveitis?
Inflammation of urea (vascular layer of eye).
87
What happens in anterior uveitis? What is a complication of anterior uveitis?
Inflammation of iris - inflamed anterior urea leaks plasma and white blood cells in the AH. Cells in the AC may settle inferiorly and cause a hypopyon (inflammatory cells in anterior chamber of eye (leukocytic exudate).
88
What is anterior uveitis seen like in the split lamp exam?
Seen in split lamp exam as a hazy anterior chamber and cells deposited in the back of cornea.
89
What is intermediate uveitis?
Inflammation of the ciliary body and leakage of cells and proteins leading to hazy vision and floats with little/no pain.
90
What is posterior uveitis?
Choroid inflammation, can spread to retina and cause blurred vision.
91
What can cause uveitis?
Isolated illness, non-infectious autoimmune cases, chronic TB, syphillis, toxoplasma, Herpes simplex, Lyme disease, CMV, associated with ankylosing spondylosis, Behcet's dx, sarcoidosis, Wegner's, systemic lupus erythematous, intraocular lymphoma, leukaemia.
92
What symptoms are associated with uveitis?
Blurry vision/vision loss (due to macular oedema due to leaky vessels), redness around cornea.
93
How do you treat uveitis?
Topical anti-inflammatories, systemic steroids, systemic immunosuppressants.
94
What does corneal abrasion result from? What symptoms does it cause?
Trauma to cornea.
Redness and pain.
Tends to heal very quickly.
95
What symptoms are experienced in pre-septal cellulitis?
Redness, lid-swelling, systemically well.
96
What can cause pre-septal cellulitis?
Lid cyst or insect bite.
97
What symptoms are experienced in orbital cellulitis?
Pain, redness, systemically unwell, diplopia, limitation in EOEM, conjunctivitis/chemosis, exophthalmos, blurred vision, fever.
98
What can cause orbital cellulitis?
Sinusitis/dental infections via haematological spread.
99
What complications can arise as a result of orbital cellulitis?
Brain abscesses and loss of vision, if not treated promptly.
100
How do you treat orbital cellulitis?
Drain fluid from eye.
101
What is multiple sclerosis?
Multiple episodes of demyelination in space and time with variable site and severity.
Involves patchy loss of myelin sheath in CNS, affecting nerve conduction, leading to progressive disability.
102
What is the pathophysiology of MS?
Autoimmune process whereby T cells cross blood brain barrier, become activated by myelin, and release cytokines (dilated BVs) and other immune cells enter past BBB and damage the oligodendrocytes.
Cytokines attract B cells (and macrophages) which produce antibodies that mark oligodendrocytes for macrophages to destroy them.
No myelin to cover nerves and scarring of nerves leads to plaques/sclera.
103
What kind of hypersensitivity is MS?
Type IV.
104
How does the body calm down the hypersensitivity reaction in MS? Why is this not effective in the long run?
Regulatory T cells come in and calm down the immune response. In early MS, re-myelination can occur.
But overtime, olgiodendrocytes die off, leading to irreversible loss of axons. Post-inflammatory gliosis may have functional deficit.
105
Which country has the highest incidence of MS in the world?
Scotland.
106
What do investigations show in MS?
Whitish plaques of demyelination and black holes, lateral cerebral atrophy may be visible.
Olgioclonal bands present in CSF (lumbar puncture) but not serum.
107
How many lesions related to one relapse?
10 lesions for every 1 relapse.
NB - scan and patients symptoms don't necessarily correlate.
108
What investigations can you use to investigate MS?
MRI, CTs, LP, visual/somatosensory evoked response, bloods, CXR.
109
What will you see on examination of someone with MS?
Afferent pupillary defect, nystagmus, abnormal eye movements, cerebellar signs, sensory signs, weakness, spasticity, hyperreflexia, plantar's extensor.
110
What are the two diagnostic criteria for MS?
Clinical - Posers criteria
MRI - MacDonald criteria
Diagnosis based on evidence of demyelination based over time and space.
111
What is Charcot's triad?
Some of the clinical signs of MS: dysarthria (plaques in brainstem), nystagmus, intention tremor.
112
What are thought to be causes of MS?
?viral (EBV), associated with autoimmune disease, genetic factors (females, HLS-DR2), fit D deficiency?, commoner in temperate climates, age of exposure.
113
What is L'hermitte's sign?
Electric shock running down back and radiating to limbs when bending the neck forward.
Can be a sign of MS, transverse myelitis, B12 deficiency etc.
114
What are common relapse symptoms in MS?
Optic neuritis, sensory symptoms (paraesthesias, numbness = plaques in sensory pathways), limb weakness, spinal cord problems (bilateral symptoms/signs +/- bladder), cranial nerve involvement, diplopia, ponsinternuclear ophthalmoplegia (e.g. vertigo, nystagmus, ataxia, upper motor neurone changes in the limb).
115
What diseases can cause similar symptoms to MS?
Neuromyelitis optica, sarcoidosis, ischaemic optic neuropathy, Wegner's granulomatosis, local compression, Leber's hereditary optic neuropathy, acute disseminated encephalomyelitis, other autoimmune diseases, e.g. SLE, vasculitis, infections, e.g. Lyme disease, HTVL-1, adrenoleucodystrophy etc.
116
What are the different types of MS? Explain how they differ.
Relapsing remitting - most common, bouts of attacks happen months/years apart, different symptoms appear and can get better or worse. Typically no increase in disability between bouts. Tend to go on to develop SPMS.
Secondary progressive (SPMS) - initially similar to RRMS but over time immune attack becomes constant - leading to steady progression in disability.
Primary progressive - constant attack on myelin (no relapses), steady increase in disability. Often presents in 50-60s with spinal and bladder problems. Poor prognosis.
Progressive relapsing - constant attack but bouts superimposed.
Sensory
Malignant
117
What do the progressive phases of MS involve?
Accumulation of signs and symptoms, e.g. fatigue, temperature sensitivity, sensory problems, stiffness/spasms, balance problems, slurred speech, swallowing, bladder and bowel problems, diplopia, oscillopia, visual loss, cognitive dementia/emotional irritability.
118
What are the first line treatments of MS?
Diet/general health
1st line Rx: beta-interferons/glatiramer acetate (reduces relapses, weekly im/sc), teriflunomide (oral), dimethyl fumigate (oral).
119
What are the side effects of first line treatment of MS?
Flu-like symptoms, injection site reactions, abnormalities of blood count and liver function.
120
What are the second line treatments for MS?
Natalizumab (reduce relapse rate, monthly infusion), fingolimoid tablets, alemtuzumab.
121
What are the ADRs for fingolimoid?
Cardiac ADRs.
122
What are the ADRs for alemtuzumab?
Autoimmune thyroid disease, good pastures/immune thrombocytopenic purpura.
123
What can be used for symptomatic treatment?
Spasticity - muscle relaxants/anti-spasmoidcs/physiotherapy
Dyseasthesia - amitriptyline gabapentin
Urinary - anti-cholingeric Rx, bladder stimulator/catheterisation
Constipation - laxatives
Sexual dysfunction, fatigue - graded exercise, mediation
Depression - CBT, meds
Cognitive - memory aids etc
Speech and swallowing - SALT
Motor impairment - MDT
124
How are acute relapses managed?
Look for underlying infection, exclude worsening of usual symptoms with intercurrent illness.
Oral prednisolone (IV), rehab, symptomatic Rx.
125
Why is it important to vaccinate MS patients?
Relapses can be associated with viral infections.
126
How does pregnancy affect relapse rate in MS?
Reduces relapses during pregnancy, increased risk 3 months post-partum.
127
What is the prognosis like in MS?
1/4 of MS patients will have their activities of daily living affected, 15% severely disabled. 1/4 will require a wheelchair at some point.
128
What are the good prognostic indicators in MS?
Females, presenting with optic neuritis, long intervals between 1st and 2nd relapse, few relapses in first 5 years.
129
What are the bad prognostic indicators in MS?
Male, older age, multifocal symptoms and signs, motor symptoms and signs.
130
What is myelitis?
Infection/inflammation of the white or grey matter of the spinal cord.
131
What are the two types of myelitis and how do they differ?
Transverse (complete) - affecting the whole width of the spinal cord.
Partial - affecting part of width of the spinal cord.
132
What is myelopathy?
Injury to the spinal cord due to severe compression. Can be cervical or thoracic normally.
133
What symptoms/signs does myelopathy cause?
Sensory and motor issues at level affected, UMN symptoms/signs below lesion.
134
What are the long tract signs in myelopathy?
******
Clonus, upgoing planters, increased tone, Hoffman sign, brisk reflexes, proprioception impairment, Romberg's test (tandem walking).
135
What is progressive multifocal leukencephalopathy?
Rare but serious demyelinating disease of the brain. It is caused by a lytic infection of oligodendrocytes by JC polyomavirus (JCV).
136
How are patients with progressive multifocal leukencephalopathy screened?
MRI annually, JC antibody blood and urine 6-monthly.
137
What are the risk factors for developing progressive multifocal leukencephalopathy?
Immunosuppressed patients (AIDS, natalizumab, dimethyl fumarate, fingolimoid).
138
What is neuromyelitis optic spectrum disorder?
Aka. Delvic disease. Very rare autoimmune disease in which there are episodes of optic neuritis and transverse myelitis.
139
What will you find in most patients with neuromyelitis optic spectrum disorder?
aquaporin-4 antibodies.
140
What symptoms do patients with neuromyelitis optic spectrum experience?
Varied - eye pain, loss of vision, reduced colour vision, limb weakness, pain in limbs, bladder, bowel and sexual problems.
141
Why do you need to be on alert for neuromyelitis optic spectrum disorder?
MS treatment can make these patients a lot worse! Despite the fact it is very rare.
NB - can be one off or relapsing.
142
How is neuromyelitis optic spectrum disorder treated?
Immunosupression.
143
What is motor neurone disease?
Aka. amyotrophic lateral sclerosis.
Occurs when motor neurones stop working properly (neurodegeneration with NO inflammation).
144
In which sex and age group is motor neurone disease most prevalent?
Men, over 60s.
145
What is the diagnosis of motor neurone disease based on?
A unique combination of UMN, LMN signs and EMG (showing lots of muscles affected and widespread denervation).
146
What are the risk factors for motor neurone disease?
Hereditary, age (40-60s), male, genetics.
147
What are the signs of motor neurone disease?
Lesion of UMN above decussation = contralateral spastic paralysis and hyperreflexia.
Lesion of UMN below decussation = ipsilateral paralysis and hyperreflexia.
Lesion of LMN = ipsilateral flaccid paralysis, areflexia, muscle atrophy.
Other signs - weakened grip, weakness in shoulder leading to difficulty raising arm, foot drop, dragging of leg, dysarthria.
148
What symptoms are associated with motor neurone disease?
Usually limb onset, lateral bulbar and respiratory involvement.
Difficulty walking, tripping/falling, weakness in legs/ankles/feet, hand weakness and clumsiness, slurred speech, trouble swallowing, muscle cramps and twitching in arms, shoulder and tongue.
149
What is an epileptic seizure?
Intermittent stereotyped disturbance of consciousness, behaviour, emotion, motor function or sensation. These can march over the body.
150
What is epilepsy?
A condition in which seizures recur, normally spontaneously.
151
Why is there a J-shaped curve in the age related incidence of epilepsy?
Commonest in babies and elderly (due to microdx) but incidence low in young adults.
152
After how many unprovoked attacks is epilepsy normally diagnosed?
2
153
How do you investigate people presenting with epileptic seizures?
MRI (esp. in <50s with possible focal onset seizures and to exclude serious cases over this age), CT (for tumours etc.), EEG (incl. with hyperventilation, photic stimulation, sleep deprivation), can do video telemetry if uncertain about diagnosis.
154
How would you investigate a first fit?
Blood sugar, ECG, consider alcohol/drugs, CT head (criteria), enquire about employment, dangerous activities.
155
What must you be sure to do in epilepsy patients?
Explain driving regulations.
156
What is thought to be the cause of epilepsy?
Abnormal neuronal discharges.
157
How is epilepsy classified?
ILAE using clinical data and EEG have separated it into two epilepsy syndromes:
Generalised seizures
Focal partial seizures
158
What are generalised seizures?
Both hemispheres of the brain affected. May begin as focal and quickly develop into generalised (known as focal onset bilateral tonic-clonic seizure). Tends to have no warning.
159
What are focal seizures?
Unpredictable brain activity localised to one hemisphere/lobe of brain. May get aura.
160
What are the different types of generalised seizure and how do they differ?
Tonic-clonic - most common, tonic phase (muscles seize up), clonic phase (muscle convulsions).
Myoclonic - short muscle twitches.
Clonic - violent muscle convulsions.
Tonic - muscles stiff/flexed. Can cause patient to fall BACK.
Atonic - muscles go floppy. Can cause patient to fall FORWARD.
Absence - impaired awareness or responsiveness.
161
What are the different types of focal/partial seizure and how do they differ?
Without impaired awareness - affects small area of brain, causes strong sensations or jerking (depending on nerves affected). Patient often remembers what happened/will know something is happening.
With impaired awareness - loss of awareness/responsiveness, usually can't remember seizure.
162
What is important to remember what classifying seizures?
Some patients (esp. those with learning difficulties) have unclassifiable seizures unique to them).
163
What are some signs of epilepsy?
Myoclonic jerks, esp. first thing in the morning, absences, strange feeling with flickering light (primary generalised epilepsy), déjà vu, rising sensation from abdomen, episodes where look blank with lip smacking, fiddling with clothes (focal onset epilepsy).
164
What symptoms can result from epilepsy?
Post-ictal confusion, paralysis of arms/legs (usually unilateral).
165
What complications can arise from epileptic seizures?
Status epilepticus.
166
What is status epilepticus?
Prolonged/recurrent tonic-clonic seizures persisting for more than 30 mins with no recovery between seizures.. Usually happens with no previous epilepsy history.
167
How is status epilepticus treated?
1st line - midazolam (buccal/intranasal, repeat after 10 mins if req), lorazepam (bolus, repeat after 20m if req), diazepam (IV/rectal, repeat after 15 min if req).
2nd line - valproate/phenotyoin infusion.
3rd line - propofol/thiopentone (anaesthetics).
168
How is epilepsy treated?
Anti-convulsants.
169
What is the first line treatment for epilepsy?
Sodium valproate, lamotrigine, levetriacetam for generalised epilepsy.
Lamotrigine or carbamazepine for partial or secondary generalised seizures.
Ethosuzamide for absence seizures.
170
What is the second line treatment for epilepsy?
Topiramate, zonisamide, carbamazepine for generalised epilepsy.
Sodium valproate, topiramate, leviteracetam, gabapentin, preganilin, lacosamide, premapanel, benzodiazepines for partial seizures.
Levetiracetam IV considered for status/near status.
171
What are the ADRs for sodium valproate?
Tremor, weight gain, ataxia, nausea, drowsiness, transient hair loss, pancreatitis, hepatitis.
172
What are the ADRs for carbamazepine?
Ataxia, drowisness, nystagmus, blurred vision, low serum sodium, skin rashes.
173
What are the ADRs for lamotrigine?
Difficulty sleeping, skin rash.
174
What are the ADRs for levetiracetam?
Irritability/depression.
175
What are the ADRs for Topiramate?
Weight loss, word-finding difficulties, tingling hands/feet.
176
What are the ADRs for zonisamide?
Bowel upset, cognitive problems.
177
What are the ADRs for lacosamide?
Dizziness.
178
What are the ADRs for pregablin?
Weight gain.
179
What are the ADRs for vigabatrin?
Behavioural problems, visual field defects.
180
What are the driving regulations for epilepsy?
After first seizure - can drive car after 6m if Ix normal, no further events. Drive HGV/PSV after 5y if Ix normal and no further events and not on anti-epileptics.
Patients can hold a group 1 license once they have been seizure free for a year/only had seizures arising from sleep for a year.
If they ever had a daytime seizure, nocturnal pattern must be established for 3 years before they can start driving (10 years and not on anti-epileptics for HGV/PSV).
181
What can cause status epilepticus?
Stroke, tumour, alcohol.
182
What is a blackout?
Transient dull or loss of vision, consciousness or memory.
183
What must you gather fro the history?
```
What they were doing at the time.
Warning feelings?
Doing the night before?
Alcohol?
Anything like this before?
How did they feel after?
Injury? Tongue biting?
Continence?
```
From witness:
Level of responsiveness, motor phenomena, PMH of head injury, birth trauma, febrile convulsions, FH.
184
What can cause a blackout?
Syncope, first seizure, concussive seizure, cardiac arrhythmias, non-epileptic attack (narcolepsy, movement disorder, migraine).
185
What are the differences between syncope and seizure?
Syncope - upright posture, seizure - any posture
Syncope - pallor common, seizure - pallor uncommon
Syncope - gradual onset, seizure - sudden onset
Syncope - injury rare, seizure - injury more common
Syncope - incontinence rare, seizure - incontinence common
Syncope - rapid recovery, seizure - slow recovery
Syncope - precipitants common, seizure - precipitants rare
186
What does vasovagal syncope result from?
Occurs in response to trigger than slows HR, blood vessels in legs widen and blood pools in the legs, which lowers BP. This reduces blood flow to the brain and causes a brief loss of consciousness.
187
What are the triggers for vasovagal syncope?
Prolonged standing, standing up quickly, trauma, venipuncture, micturition (fainting shortly after urination), long coughing fit.
188
What symptoms can some experience before a vasovagal syncope?
Predome (light headed, nausea, hot, sweating, tinnitus, tunnel vision).
189
If you stand up too quickly after a vasovagal syncope what can happen?
Risk of fainting again.
190
How can you prevent a vasovagal syncope if you feel it coming on?
Lie down and raise legs in the air.
191
What causes a hypoxic seizure?
Occurs when patients are kept upright during a faint, e.g. in aircrafts, at the dentists, people meaning well. Seizure-like activity may occur.
192
What causes a concussive seizure?
A blow to the head.
193
What heart problems can seizures cause?
Cardiac arrhythmias.
194
When would you want to be considering if there is FH of sudden death or cardiac history?
If a young person is collapsing during exercise.
195
What are non-epileptic attacks?
May resemble tonic-clonic seizure, involve swooning or bizarre movements. Can be frequent and prolonged. May have history of medically unexplained symptoms/history of drug abuse.
196
What are primary headaches? What sort of headaches fall into this category?
No underlying medical cause. Includes tension type headache, migraine and cluster headaches.
197
What are secondary headaches?
These have identifiable structural or biochemical cause (e.g. tumour, meningitis, vascular disorders, systemic infection, head injury, drug-induced). Not all are sinister.
198
What are tension type headaches? What can cause these?
Milk-bilateral headache which is often tight/pressing. No significant associated features and not worsened by routine physical activity. Life-time prevalence almost 50%. Stress, squinting, poor posture, tiredness, dehydration, missing meals, lack of exercise, bright sun light, noise...
199
What are the types of tension type headaches?
Infrequent ETTH <1 day/month, frequent ETTH 1-14 days/month, chronic TTH 15+ days/month.
200
How do you treat a tension type headache?
Aspirin/paracetamol, NSAIDs (limit to 10 days/month to avoid MOH). Preventative Rx: tricyclic antidepressants (amitryptline, dopthiepine, nortriptyline; rarely req).
201
What is a migraine?
Complex chronic disorder with episodic attacks involves integrated brain mechanisms and CNS structures (cortex, brainstem, trigeminal system, meninges).
The brain of the migraineur is hypersensitive to stimuli. could be to do with a number of things (insufficient cortical inhibition, reduced pre-activation of sensory cortices as a result of thalamocortical drive).
202
Give examples of migraine triggers.
Normal life events (diet, environmental stimuli, low oestrogen in women, stress, hunger, dehydration, sleep distruabcnce.
203
What symptoms are associated with migraine?
Throbbing unilateral headache, photophobia, photophobia, nausea.
Symptoms between attacks incl. enduring predisposition to future attacks, anticipatory anxiety.
204
What is an aura with migraine?
33% of migraineurs get this.
Transient neurological symptoms resulting from brainstem/cortical dysfunction. May involve visual, sensory, motor or speech systems. Slow evolution of symptoms. Duration: 15-60 minutes. Can be confused with TIA.
205
What is a visual aura in migraine?
Most common. Involves loss of vision (e.g. scotoma), a blind spot that spreads across the visual field, or loss of vision in one field (hemianopia less common).
206
What is a sensory aura in migraine?
Paraesthesia (tingling) typically starts in one hand, spreading to arm, elbow, face, lips and tongue.
207
What is a motor aura in migraine?
Typically experienced on one side, affecting the hand and arm.
208
What other rare type of aura can you get in migraine? (aside from motor, sensory and visual).
One affecting the brainstem, results in quadriplegia, loss of consciousness or cranial nerve deficits.
209
How are migraines treated?
Aspirin/NSAID, triptans (use less than 10x/month to avoid MOH).
Prophylaxis - propanolol, candesartan, anti-epileptics (topiramate, valproate, gabapentin), tricyclic antidepressants (amtitryptiline, dothiepin, nortryptiline), venaflaxine.
210
What are the 5 migraine phases?
Premonitory phase (70%) - mood alterations, muscle pain, cravings, cognitive changes, fluid retention, yawning.
Aura phase
Early headache phase - mild pain without associated migraine symptoms.
Advanced headache phase - moderate to severe pain with associated symptoms (nausea, photophobia, phonophobia, disability).
Postdrome - migraine associated symptoms beyond the resolution of the headache (can last 1-2 days).
211
What is believed to cause the aura phase of migraines?
Believed to arise from electrical disturbance called cortical spreading depression.
212
What do you call migraines that involve auras not followed by a headache?
Acephalgic migraines.
213
What is a classed as a chronic migraine?
Headache on 15+ days of the month, more days have to be migraine than headache, for more than 3 months.
214
What is a transformed migraine?
History of episodic migraine, increasing frequency of headaches over weeks/months/years, migrainous symptoms become less frequent/severe.
Many patients have episodes of severe migraine on background of less severe featureless frequent/daily headache.
Transformation can occur w or w.o escalation in medication use.
215
How does taking patients with medication overuse headaches off their medication help affect their headaches?
Usually dramatically improves headache frequency.
216
What is a medication overuse headache?
Headache present on 15+ days/month which has developed or worsened whilst taking regular symptomatic medication. Can occur in any primary headache, migraineurs particularly prone (even if taking meds for another reason).
217
What drugs can cause medication overuse headache?
Triptans, ergots, opoids and combination analgesics more than 10 days/month.
Simple analgesics >15 days/month.
Caffiene overuse can also cause MOH.
218
Does migraine without aura get better or worse during pregnancy?
Better usually.
219
Does migraine with aura get better or worse during pregnancy?
Usually doesn't change.
220
Can the first migraine occur during pregnancy?
Yes (usually with aura).
221
What is contraindicated if patients have migraine with aura?
Combined OCP contraindicated in migraine with aura (okay if no attacks for more than 5 years, stop in aura recurs).
222
What sort of drugs must you avoid in the pregnant or women of childbearing age?
Anti-epileptics.
| Or must counsel about teratogenicity and ensure contraception.
223
What medications are okay to use for headaches in the pregnant?
Paracetamol or preventative propanolol or amitriptyline.
224
What are trigeminal autonomic cephalgias?
Primary headaches with a common clinical phenotype consisting of trigeminal pain with autonomic signs.
225
What types of trigeminal autonomic cephalgias are there?
Cluster Hedaache
Paroxysmal hemicranias
SUNCT (short lasting unilateral neuralgiform headache with conjunctival injection and tearing)
SUNA (short lasting unilateral neuralgiform headache with autonomic symptoms
226
What sort of symptoms do you get in trigeminal autonomic neuralgias?
Unilateral severe head pain (mostly V1), cranial autonomic symptoms, incl. conjunctival injection/lacrimal, nasal congestion/rhinorrhea, eyelid oedema, forehead/facial sweating, miosis/ptosis (Horner's).
Attack frequency and duration differ.
227
What often do attacks occur in cluster headache?
1-8 per day.
228
What often do attacks occur in paroxysmal hemicrania?
1-40 per day.
229
What often do attacks occur in SUNCT?
3-200 per day.
230
How long do attacks tend to last in cluster headache?
15-180 mins.
231
How long do attacks tend to last in paroxysmal hemicrania?
2-30 mins.
232
How long do attacks tend to last in SUNCT?
5-240 secs.
233
What sort of pain is experienced in cluster headache?
Sharp and throbbing.
234
What sort of pain is experienced in paroxysmal hemicrania?
Sharp and throbbing.
235
What sort of pain is experienced in SUNCT?
Stabbing pain.
236
How intense is the pain in the trigeminal autonomic cephalgias?
Very severe.
237
What is the circadian periodicity like in cluster headache, paroxysmal hemicrania, and SUNCT?
Cluster headache- 70% - attacks tend to occur at same time each day.
Paroxysmal hemicrania - 45%
SUNCT - absent
238
Why is cluster headache so called?
Episodic in 80-90%. Attacks tend to cluster into bouts typically lasting 1-3 months with periods of remission lasting at least 1 month.
239
What is a chronic cluster headache?
10-20% have this. Bouts last <1y without remission or remission lasts less than 1 month.
240
What can trigger attacks in cluster headaches?
Alcohol during a bout but not in remission.
241
What symptoms are experienced in cluster headache?
Pain mainly orbital and temporal. Rapid onset (max within 9 mins in most) with rapid cessation of pain. Very severe. Patients restless and agitated during an attack. Prominent ipsilateral autonomic symptoms, migranous symptoms, premonitory symptoms, nausea, vomiting, photophobia, phonophobia, typically aura.
242
How are cluster headaches treated?
s/c sumatriptan 6mg or nasal solmatriptan 5mg
100% oxygen 7-12L/min via tight fitting non-rebreathing max is effective.
Headache bout - occipital depomedrone injection (same side as headache) or tapering course of oral prednisolone.
Preventative - verapamil (high dose), lithium, methysergide (risk of retroperitoneal fibrosis), topiramate.
243
What are the two different types of paroxysmal hemicrania?
80% have chronic, 20% have episodic.
244
What are 1/10th of attacks be precipitated by?
Bending/rotating of the head.
245
What are the signs/symptoms of paroxysmal hemicrania?
Sudden onset of excruciating orbital/temporal unilateral pain. Half of people are restless and agitated during an attack. Prominent ipsilateral autonomic symptoms, migrainous symptoms may be present. Background continuous pain can be severe.
246
How is paroxysmal hemicrania treated?
No abortive Rx.
Prophylaxis with indomethacin (alternatives: COX-II inhibitors, topiramate).
247
What triggers trigger a SUNCT attack?
Wind, cold, touching, chewing.
248
What symptoms are experienced in a SUNCT attack?
Unilateral, suprobital or temporal stabbing or pulsating pain, conjunctival injection and lacrimation.
249
How is SUNCT/SUNA treated?
No abortive Rx.
Prophylaxis - lamotrifine, topiramate, gabapentin, carbamezapine/oxccarbazepine.
250
What is trigeminal neuralgia?
Paroxysmal attacks of laminating pain. More common in middle age/elderly.
251
What causes trigeminal neuralgia?
Vascular loop causing compression of fifth cranial nerve in posterior fossa (BV rubs on nerve and irritates it).
252
What can trigger an attack of trigeminal neuralgia?
Wind, cold, touch, chewing.
253
What symptoms are experienced in trigeminal neuralgia?
Unilateral maxillary or mandibular pain > ophthalmic division. Stabbing pain lasting 5-10s. Attack frequency similar to SUNCT but has refractory period (unlike SUNCT), autonomic features also common.
254
How is trigeminal neuralgia treated?
No abortive Rx.
Prophylaxis: carbamazepine, oxcarbamezepine.
Surgical intervention: glycerol ganglion injection, stereotactic radiosurgery, decompressive surgery.
255
What kinds of presentation would be indicative of sinister causes of secondary headache?
Associated with head trauma, first or worse headache, sudden (thunderclap headache) onset, new daily persistent headache, change in headache pattern or returning patient headache.
256
What are the red flags for patients presenting with headache?
```
New onset
New or change in headache and over 50
Immunosupressed/cancer patient
Change in headache frequency
Characteristics/associated symptoms
Focal neurological symptoms
Non-focal neurological symptoms
Abnormal neurological examination
Neck stiffness
High pressure
Headache worse lying down
Wakening the patient up
Precipitated by physical exertion/Valsalva
Risk factors for cerebral venous sinus thrombosis
Low pressure (headache precipitated by sitting/standing up)
GCA (jaw claudication or visual disturbance)
Prominent or beaded temporal arteries
```
257
What can cause a thunderclap headache?
PRIMARY: migraine, primary thunderclap headache, primary exceptional headache, primary headache associated with sexual activity
SECONDARY: SAH, intracerebral haemorrhage, TIA, stroke, carotid/vertebral dissection, cerebral venous sinus thrombosis, meningitis, encephalitis, pituitary apoplexy, spontaneous intracranial hypotension.
258
What symptoms are experienced during a thunderclap headache?
High intensity headache reaching maximum intensity i less than a minute. Majority peak instaneously.
259
From where do aneurysms tend to arise in the brain?
Circle of Willis.
260
What is the common presentation of SAH?
1 in 10 patients with thunderclap have a SAH.
261
What mostly causes SAHs?
85% aneurysmal.
262
How do you investigate a patient that has presented with a thunderclap headache?
All patients presenting with sudden, severe headache that peaks within a few mins and lasts less than an hour, even if exam normal, must consider SAH. Same day hospital appointment - history, exam, CT brain, LP (must be done in >12 hours after headache onset). CT +/- LP unreliable after 2wks, angiography must be done after this time.
Exclusion is by absence of blood products in CSF.
263
What are the risk factors for SAH?
Smoking (3x), high blood pressure.
264
What are the possible complications of SAH?
Hydrocephalus, vasospastic infarcts, disability.
265
How do you manage a SAH?
If ruptured aneurysm: endovascular coil embolisation, surgical clipping, treatment of complications.
Note - 50% mortality, 20% survivors remain dependent.
266
Why is raised intracranial pressure a problem?
Cranium is hard, rigid and closed. For pressure to be stable, there must be the correct amount of brain tissue/blood/CSF. The brain cannot go through the falx (but can go around it --> herniation), it cannot go through the skull (but can go round foramen magnum --> coning).
267
How do you monitor patients with raised ICP?
Brain tissue oxygenation monitoring (probe to monitor oxygenation of tissue, detect and treat low oxygenation to increase CPP), micro-dialysis (Ix brain metabolism, implantation of specially designed catheters to collect small molecular weight substances to help measure neurotransmitters, peptides and other substances).
268
How is motor neurone disease managed?
Supportive - PEG, NIV, physiology, OT, care.
Riluzole (prolongs survival by 2 months).
269
How does riluzole work in treating motor neurone disease?
It is an anti-glutamate that blocks the effects of glutamate. Glutamate is one of the things that causes nerve damage as excess glutamate over-stimulates nerves.
270
What is the life expectancy in motor neurone disease?
3-5 years from symptom onset.
Most die as a result of respiratory failure.
Its an incurable disease.
271
What is brown-sequard syndrome a result of?
Damage to one half of the spinal cord.
E.g. - herniated disc, tumour, trauma, ischaemia or infection disrupting pyramidal, spinothalamic, posterior column tracts on affected side.
272
What symptoms are experienced in Brown-Sequard syndrome?
Paralysis, loss of proprioception on same side as lesion, loss of pain, temperature sensation on contralateral side of lesion.
NORMAL sensation above level of lesion.
273
What is kinetosis?
Aka. motion sickness.
Powerful, maintained stimulation of the vestibular system can give rise to this. More likely to happen if the visual and vestibular inputs to cerebellum are in conflict. Cerebellum generates sickness signal to hypothalamus to bring about ANS changes.
274
What are the symptoms of kinetosis?
Nausea/vomiting, reduced BP, dizziness, sweating, pallor (ANS symptoms).
275
What is thought to cause menieres disease?
Over production of endolymph leading to increased pressure.
276
What are the symptoms of menieres disease?
Vertigo, nausea, nystagmus, tinnitus.
277
What drugs can damage the inner ear?
Streptomycin, gentamicin.
278
What sort of lesions cause nystagmus at rest?
Brainstem lesions.
279
What can cause raised ICP?
```
Cerebral oedema (can be post-traumatic)
Space occupying lesions - tumours (e.g. glioblastoma multiforme), cerebral abscess, venous infarct with focal area of haemorrhage, meningioma, hydrocephalus, papilloedema, abscesses.
```
280
What are the signs associated with raised ICP?
Pupillary dilatation (stretch on Cr. N III - uncal herniation tends to put pressure on it), brainstem death due to coning, 6th nerve palsy, cognitive impairment, focal symptoms/signs, non-focal symptoms/signs, e.g. personality changes, drowsiness/altered consciousness, CSF obstruction can present with hydrocephalus).
281
What symptoms are associated with raised ICP?
Progressive headache (worse in morning/waking patient from sleep), visual obscurations, diplopia, blurred vision, pulsatile tinnitus, nausea, vomiting, somnolence.
282
What complications can result from raised ICP?
Herniation between the intracranial spaces, leading to ischaemia.
283
What are the different types of herniations that can result from raised ICP?
Right to left
Left to right
Uncal/tentorial - cerebellum moves inferiorly over edge of tentorium crushing cerebral aqueduct
Subfalcine - midline shift (cingulate gyrus pushed over to side and herniates under falx, crushing ventricle and displacing it downwards)
Coning - cerebellum moves into foramen magnum
Cerebellar tonsillar herniation - causes brainstem
284
How is raised ICP treated?
Head end elevation to facilitate venous return, osmotic agents (e.g. mannitol/hypertonic saline), hyperventilation (decrease CSF), barbiturate coma (decrease cerebral metabolism/coronary blood flow, maintain good PO2), surgical decompression.
285
What is a haematoma?
A localised area of haemorrhage.
286
Where do intracranial tumours tend to be in adults?
Above the tentorium.
287
Where do intracranial tumours tend to be in children?
Below the tentorium.
288
How do you investigate a suspected intracranial tumour?
History, examination, CXE, abdomen/pelvis/chest CT, mammography, biopsy skin lesions/lymph nodes (thinking of secondary sources), CT, mRI, biopsy, angiography if haemangioblastoma suspected.
289
What are the signs of a intracranial tumour?
Tumour close to/in CSF pathway (esp. posterior fossa) tend to block cerebral aqueduct or median/lateral apertures (esp. in children) causing hydrocephalus.
290
What symptoms are associated with intracranial tumours?
Focal neurological deficit (e.g. hemiparesis, dysphagia, hemianopia, cognitive impairment, cranial nerve palsy, endocrine disorder).
If on precentral gyrus weakness can indicate where lesion is.
If close to 4th ventricle can press on vomiting centre - N&V.
Presence of tumours above tentorium can focal/generalised epilepsy (1st fit - 20% tumour)
291
What is Gerstmann Syndrome?
Results from damage to the inferior parietal lobule of the dominant (left) side - it gives a typical set of symptoms (dysgraphia/agraphia, dyscalculia/acalculia, finger agnosia/anomia, left-right disorientation.
292
How are intracranial tumours treated?
Accurate tissue diagnosis, corticosteroids (dexamethasone), treat epilepsy (anti-convulsants), analgesics, anti-emetics, consuelling, radiotherapy, surgery, chemotherapy, endocrine replacement.
293
What are the various types of benign malignant primary intracranial tumours?
Those resembling glial cells - glioblastoma, astrocytoma, oligodendrolioma, ependymoma.
Childhood malignant tumour - medulloblastoma (resembles embryonic neural cells).
294
What are the types of benign intracranial tumours?
Meningioma (from arachnoidal cells)
Schwannoma, neurofibroma (from nerve sheath cells)
Adenomas (of the pituitary gland)
Lymphoma (from lymphoid cells)
Haemangioblastomas (from capillary vessels)
295
What sort of appearance to gliomas take on?
Diffuse edges, not encapsulated, malignant but do not metastasise outside the CNS.
296
What different types of astrocytes can you get?
Low grade astrocytoma (bland cells, similar to normal astrocytes, slow growing small cells with single nuclei).
Glioblastoma multiforme (most malignant astrocytoma, high grade, large tumour with necrosis, cellular, atypical, fast growing. Cells of tumour are large with multiple/irregular nuclei, this cancer spreads through the white mater and CSF pathway.
297
How do you treat a glioblastoma multiforme?
Complete surgical excision impossible, biopsy or debunk only, steroids, anticonvulsants, radiotherapy, chemotherapy (temazolamide).
298
What is the life expectancy with glioblastoma multiforme?
1 year.
299
What are meningiomas? How do they present?
From arachnocytes (cells that make the covering of brain). Benign, do not metastasise, but can be locally aggressive). Slow growing, extra-axial, often resectable.
Presentation - headache.
300
Where do meningiomas most commonly occur?
Along the falx, convexity, or sphenoid bone.
301
What is the appearance of meningiomas microscopically?
Bland cells forming groups sometimes with calcification.
Small groups of cells whorl around each other resembling an arachnoid granulation.
NB - calcification sometimes known as a psammoma.
302
How is meningioma treated?
Usually cured by surgery.
303
What is 8th vestibulochcochlear nerve schwannoma sometimes called? Where does this tend to occur and how does it present?
Acoustic neuroma. At angle between pons and cerebellum. Unilateral deafness. NB - it is benign.
304
What do adenomas of the pituitary gland often secrete?
Pituitary hormones.
305
How can pituitary adenomas present?
Visual disturbance (if impinges on optic chiasma), hormone imbalance.
306
What are lymphomas? What issues are there with treatment of lymphoma of the brain?
High grade neoplasms, usually diffuse large B cell lymphoma. Often deep and central site in brain (difficult to treat and biopsy). Generally doesn't spread out CNS.
Difficulties in Rx - deep and central site, drug doesn't cross BBB.
307
What are haemangioblastomas?
Tumours of the BVs, space occupying and may bleed (often into the cerebellum)
308
Why should you perform a lumbar puncture on a patient with a suspected IC mass?
DO NOT perform a LP on a patient with a suspected intracranial mass, as you could cause a herniation syndrome which could be fatal.
309
From where do cerebral metastases most commonly arise?
Breast, lung, kidney, colon, melanoma (mostly carcinomas).
NB - histological status identical to primary.
310
Where do brain tumours mostly commonly metastasise to?
Liver, lungs.
311
How common are cerebral mets?
15-30% of cancer patients get them. But rare in children.
312
How are cerebral mets treated?
Steroids, anti-convulsants, radiotherapy (whole brain, stereotactic), surgery.
If more than one lesion, radiotherapy is best.
313
How must you investigate if there is intracranial hypotension?
MRI brain and spine.
314
What can cause intracranial hypotension?
Spontaneous, iatrogenic (post LP), dural CSF leak.
315
What symptoms are associated with intracranial hypotension?
Headache (w. postural component, develops/worsens soon after assuming upright position, resolves/lessens after lying down).
Once headache becomes chronic, it loses its postural component.
316
How is intracranial hypotension treated?
Bed rest, fluids, analgesia, (IV) caffeine, blood patch.
317
What is giant cell (temporal) arteritis?
Arteritis of large arteries (on spectrum with polymyalgia rheumatic).
318
In which patients should you always consider giant cell arteritis?
Any patient over 50 with new headache.
319
How do you investigate giant cell arteritis?
Elevated ESR supports diagnosis, raised CRP, raise platelet count. If suspected - temporal artery biopsy.
320
What signs are associated with giant cell arteritis?
Prominent, beaded/enlarged temporal arteries may be present.
321
What symptoms are associated with giant cell arteritis?
Headache usually diffuse, persistent and may be severe, may be systemically unwell, scalp tenderness, jaw claudication, diplopia, neck pain, nausea/anorexia, febrile symptoms and pain over temples.
322
How is temporal arteritis treated?
High dose of prednisone (2y course).
323
Why is it so important to treat temporal arteritis quickly?
It can develop in other structures, e.g. aorta, coronary, mesenteric arteries and this may be fatal.
324
What is hydrocephalus?
Excess CSF within intraventricular spaces causing dilatation of the ventricles.
325
What are the two types of hydrocephalus and how do they differ?
Communicating hydrocephalus (aka non-obstructive) - CoH - CSF free to move around ventricles, no blockage in the ventricular system.
Non-communicating hydrocephalus (aka obstructive hydrocephalus) - NCH - CSF can't move freely from start to finish, there is a blockage.
326
What causes communicating hydrocephalus?
Mostly due to problems with CSF resorption, i.e. resorption < production.
Very rarely choroid plexus papillomas have been known to present in this way. Consider infection (after bacterial meningitis most commonly), subarachnoid haemorrhage (blood and blood breakdown products cause scarring of arachnoid granulations), post-op, trauma etc.
327
What causes non-communicating hydrocephalus?
```
Physical obstruction to normal flow of CSF. Could be:
Aqueductal stenosis
Tumours
Masses
Cysts
Infection
Haemorrhage/Haematoma
Congenital malformations
```
Most common: Aqueductal stenosis at Foramen of Munro
328
What are the signs and symptoms of communicating hydrocephalus?
Children - (unfused sutures) disproportionate increase in head circumference, failure to thrive.
Fused sutures - papilloedema, gait disturbance, 6th nerve palsy, up gaze difficulty etc.
Symptoms - headache, nausea, vomiting
329
What are the signs and symptoms of non-communicating hydrocephalus?
Processes that have developed over long period of time usually cause gradual development of symptoms. Acute processes, e.g. bleed, can cause acute obstruction with rapid mental decline.
330
What complications are associated with communicating hydrocephalus?
Sudden dramatic incline in CSF respiration ability. Medical emergency. Neurological decline can be rapid (patients can go from sleepy to obtunded to requiring intubation).
331
How is communicating hydrocephalus treated?
EVD (+ shunt replacement in most).
Ventriculo-peritoneal most common, lumbar peritoneal sometimes used though over drainage is a problem. Ventriculo-atrial can be considered in cases of peritoneal failure?????
332
What are the earliest signs of hydrocephalus?
Dilated temporal horns of lateral ventricles (normally invisible), then third ventricle balloons and lateral ventricles increase in size, then peripheral sulci also become affected.
333
What issues can arise with treated? How are these managed?
qShunt infections
Rx - antibx +/- surgery
334
How is hydrocephalus treated?
Surgery.
Acute - emergency placement of external ventricular drain (EVD) catheter passed through patients scalp (bore hole) into third ventricle to drain CSF.
335
What is the problem with EVD in treatment of hydrocephalus?
EVD cannot be maintained indefinitely (if patients cannot tolerate clamp/weaning of EDV prior to removal, permanent shunt may be needed).
High rate of infections with EVD - must remove ASAP.
336
What is Evan's ratio?
Ratio of maximum width of anterior horns of the lateral ventricles to the maximum width of the Calvarium at the same level as the foramen of Munro.
Ratio greater than 0.3 defines ventriculomegaly.
337
What is the ventricular index?
Distance between the falx and the lateral wall of the anterior horn in the coronal plane at the level of the foramen of Munro.
Should be less than 50%.
338
What are the reasons that can lead to damage of the cranial nerves?
May be damaged within the brain, e.g. ischaemia/tumour.
Or crossing the subarachnoid space, e.g. meningitis.
Or outside the skull, e.g. by base of skull tumours arising in nasopharynx.
339
What is optic neuritis?
Demyelination within the optic nerve, which also causes inflammation around the eye muscles.
340
How can you investigate optic neuritis?
Visual evoked tracing - can look at optic nerve response to stimuli.
In optic neuritis - will see delay and flattening of the curve, meaning the nerve is working slower.
341
What is optic neuritis commonly the first sign of?
MS.
342
What signs are associated with optic neuritis?
Optic disc swelling (retrobulbar swollen disc may look normal), relative afferent pupil defects.
343
What symptoms are associated with optic neuritis?
Gradual onset of monocular visual loss, central scotoma, reduced colour vision (red desaturation), reduced visual acuity, pain on eye movement. Can be asymptomatic.
344
What are the possible complications of optic neuritis?
Optic atrophy can be seen later.
345
How is optic neuritis managed?
Mostly settles itself over a couple of weeks.
346
Name 6 eye movement disorders.
```
Isolated third nerve palsy
Isolated fourth nerve palsy
Isolated sixth nerve palsy
3/4/5 nerve palsy combination
Supranuclear gaze palsy
Nystagmus
```
347
What can cause an isolated third nerve palsy?
Diabetes, hypertension (painless, pupils spared), posterior communicating artery aneurysm, raised ICP (painful, pupil affected).
348
What can cause an isolated sixth nerve palsy?
Idiopathic (most common in young, overweight people), diabetes, meningitis, raised ICP.
349
What is nystagmus?
A condition of involuntary eye movement.
350
What causes nystagmus?
Can be congenital, resulting from peripheral vestibular problem, central/brainstem disease, cerebellar disease or toxins (e.g. medications - carbamezapines and alcohol).
351
What are the complications of nystagmus?
Can cause serious visual impairment.
352
What is supra nuclear gaze palsy?
good question...
353
What is Bell's palsy?
aka. idiopathic facial nerve palsy. Lower motor type.
354
What are the symptoms of Bell's palsy?
Unilateral weakness which develops within 2 days (dropping of eyelid, corner of mouth, drooling, dry mouth, loss of taste, irritation to eye). Often preceded by pain behind the ear.
355
What are the complications of Bell's palsy?
Eye closure affected leading to risk of corneal damage which may lead to corneal ulcers and loss of vision.
356
How is Bell's palsy managed?
Steroids, eye drops to stop eyes doing out (refer to ophthalmologist).
357
What can you do for patients who do not fully recover from Bell's palsy?
80% fully recover within 3 months. If not - refer to speech and language and for cosmetic surgery (Botox and surgery).
358
What is the different between UMN paralysis and LMN paralysis?
In bilateral UMN paralysis can still move forehead. In LMN lose ability to move forehead.
359
What can cause a LMN paralysis?
Bell's palsy.
360
What can cause a UMN paralysis?
Stroke or tumour.
361
What is vestibular neuritis?
Infection/inflammation of the vestibular nerve in the inner ear.
362
What causes vestibular neuritis?
?Viral (may have started from cold/flu).
363
What symptoms are experienced in vestibular neuronitis?
Very sudden onset of disabling vertigo, violent vomiting.
364
How is vestibular neuronitis treated?
Anti-emetics and bed rest.
365
What is dysarthria?
Disorientated articulation, slurring of speech.
366
What is dysphagia?
Difficulty swallowing.
367
What is bulbar palsy?
Signs/symptoms linked to impaired function of cranial nerves Ix, X, XI, XII which occurs due to lower motor lesion in the medulla oblongata or from lesion of the lower cranial nerves outside the brainstem.
368
What can cause bulbar palsy?
Lower motor lesion - e.g. NMD, polio, tumours, vascular lesions of the medulla, syphillis.
369
What are the signs of bulbar palsy?
Tongue fasiculations, nasal speech, abnormal/absent jaw jerk reflex, absent gag reflex.
370
What symptoms are experienced in bulbar palsy?
Nasal regurgitation, dysphagia, difficulty chewing, dysarthria.
371
What is pseudobulbar palsy?
Characterised by inability to control facial movements (e.g. chewing, speaking).
372
What causes pseudobulbar palsy?
Upper motor lesion - e.g. vascular lesions of both internal capsules, MND.
373
What signs are associated with pseudobulbar palsy?
Spastic, immobile tongue, brisk jaw jerk, brisk gag reflex.
374
What symptoms are associated with pseudobulbar palsy?
Dysarthria, dysphonia, dysphagia.
375
What is a stroke?
Sudden onset of focal or global neurological symptoms caused by ischaemia or haemorrhage and lasting more than 24 hours.
In stroke, there is irreversible ischaemia leading to localised brain death.
376
What are the two types of stroke? Of these, which is most common?
85% strokes are ischaemic (blocked BVs denying brain oxygen and nutrients).
15% of strokes are haemorrhagic.
377
75% of strokes occur in which group of people?
Over 75ys.
378
How do you investigate stroke?
Diffusion weighted MRI (sensitive), but tend to have to do Xray as patients too ill.
Routine bloods (FBC, glucose, lipids, ESR...), CT, MRI headscan, echocardiogram (?AF, LVH, valves, ASD, VSD, PFO), carotid doppler US (?stenosis), cerebral angiogram/venogram (vasculitis?), hyper-coagulable blood screen.
379
What other conditions could you consider if someone presents with stroke-like symptoms?
Post-ical states (e.g. Todd's paralysis), hypoglycaemia, intracranial masses, vestibular disease, Bell's palsy, functional hemiparesis, migraine, demented patients with UTIs.
380
What are the three broad categories of things that can happen to blood vessels to cause a stroke?
Vessel wall changes - e.g. abnormality of wall, atheroma or vasculitis, outside pressure (strangulation, spinal cord compression, compression of veins).
Blood flow and pressure changes - e.g. decreased blood flow, increased blood pressure bursting vessels.
Blood constituents - e.g. thrombosis of arteries and rarely veins, bleeding due to anticoagulation, reduced platelets and clotting factors.
381
What are the common causes of ischaemic stroke?
Large artery atherosclerosis, e.g. carotid (most common)
Cardioembolic, e.g. AF, recent MI, atrial myxoma
Small (lacunar) artery occlusion
Cryptogenic
382
What are some rare causes of ischaemic stroke?
```
Arterial dissection
Venous sinus thrombosis
Hypercoagulable state
Giant cell arteritis
Artery to artery embolism
```
383
What are the types of haemorrhagic stroke?
```
Primary intracereberal haemorrhage
Secondary haemorrhage (SAH, arteriovenous malformation)
```
384
In practice, what are the three main causes of localised interrupted blood supply leading to stroke?
1. atheroma and thrombosis of artery causing ischaemia - (common at carotid bifurcation), atheroma causes some narrowing, thrombosis (platelet + fibrin) results in severe narrowing. ICA thrombosis, typically get ischaemia in MCA territory.
2. thromboembolism (e.g. from L atrium) causing ischaemia - thrombosis of atrial appendages more common in AF - can embolise to aorta, then carotids and other arteries (e.g. MCA).
3. Ruptured aneurysm of cerebral vessel causing haemorrhage (beyond vertebral arteries and carotids, cerebral arteries thin walled. Weakening of wall can cause aneurysms, which can rupture (esp. in severe hypertension). Leads to decreased blood flow distally to brain due to spasm of artery and haemorrhage.
385
In practice, what are the three main causes of generalised interrupted blood supply/hypoxia?
1. low oxygen in blood (e.g. carbon dioxide poisoning, near drowning, respiratory arrest)
2. inadequate supply of blood (blood flow disrupted), e.g. cardiac arrest, hypotension, brain swelling (e.g. trauma)
3. inability to use oxygen, rare, e.g. cyanide poisoning
386
What are the two common sites of ruptured vessels causing haemorrhagic strokes?
1. basal ganglia - microaneurysms form in hypertensive patients
2. circle of Willis - berry aneurysms form in the hypertensive patient
387
What is the prognosis in stroke?
1/3rd die within 1y. 50% survivors remain dependent.
388
What is atrial myxoma?
Benign tumour in atrium of heart. Most often grows on atrial septum.
389
What are ischaemic strokes?
Failure of cerebral blood flow to part of the brain. Brain v. sensitive to ischaemia - few mins hypoxia/anoxia --> ischaemia --> infarction --> necrosis. Neuronal damage is permanent.
Further damage can result from oedema (depending on size/location of stroke).
390
What is a penumbra in relation to ischaemic stroke?
Part of brain dies regardless of what you do, penumbra can be saved. The quicker you act, the more penumbra can be saved.
391
What is haemorrhagic transformation in terms of ischaemic stroke?
Secondary haemorrhage into the stroke.
392
What will you normally see when you investigate ischaemic stroke patients?
Wedge-shaped area of localised brain death (regional cerebral infarct) reflecting arterial perfusion territory. This is soft and then becomes cystic.
393
Why is the location of the stroke so important?
Location is so important and even small areas of necrosis can be devistating because of where they are.
394
What are the risk factors for ischaemic stroke?
Hypertension (biggest), smoking (x2), cholesterol (increased serum lipids --> excessive LDL within arterial wall), HTN, smoking, diabetes, diet, high BMI (risk for vascular stroke), lack of exercise, heavy drinking, previous stroke, old age, male, HF of stroke, impaired cardiac function, OCP/HRT (oestrogen), hyper coagulable states (malignancy, genetic).
395
Why does hypertension increase your risk of stroke?
Chronic HTN worsens atheroma, affect lacunes (small distal arteries) as this is where pressure is already greatest. Lowering BP lowers risk of stroke.
396
Why must you be careful of aggressively lowering cholesterol?
Can increase risk of haemorrhagic stroke.
397
How can you BE FAST to notice a stroke?
B - balance
E - eyes
F - face
A - arm weakness
S - speech problems
T - time to call 999
Only have 3-6 hours to make a difference and protect vulnerable tissue.
398
What drugs can you use to treat ischaemic stroke?
tPA (tissue plasminogen activator) - ONLY if 4.5h from symptom onset, disabling neurological deficit, symptoms present for longer than 60 mins and consent obtained.
399
What procedures can be used to treat stroke?
Thrombectomy/intra-arterial tPA - fishing out the clot could reduce death/disability and improve outcomes.
400
What are the contraindications for using tPA?
```
Anything increasing risk of haemorrhage -
Blood on CT
Recent surgery
Recent episodes of bleeding
Coagulation problems
BP >185 systolic or >110 diastolic
glucose <2.8 or >22mmol/L.
```
401
Benefit from tPA halves every....
90 minutes.
402
What is involved in secondary prevention of ischaemic stroke?
Anti-hypertensives, anti-platelets, lipid lowering agents, warfarin for AF, rehab, carotid endarectomy.
403
What are the different types of stroke?
Total anterior circulation stroke (TACS) - usually due to MCA damage
Partial anterior circulation stroke (PACS)
Lacunar stroke (LACS)
Posterior circulation stroke (POCS)
404
What symptoms are experienced in a TACS?
Can have motor and sensory symptoms in arms/legs/face.
| Hemianopia + dysphagia/neglect.
405
What symptoms are experienced in a PACS?
Can have motor and sensory symptoms in arms/legs/face.
| Can have hemianopia/dysphagia/neglect.
406
What symptoms are experienced in POCS?
Can have motor and sensory symptoms in arms/face/legs. Can have hemanopia/dysphagia/neglect. Will have brainstem and/or cerebellar signs.
407
Rank the stroke types in terms of their death/dependence at 6mnths (worse --> best).
TACS, PACS, LACS, POCS.
408
Lacunar strokes are devoid of what sort of symptoms?
Cortical signs, e.g. no dysphagia, neglect, hemianopia.
409
What are the different types of lacunar strokes?
Pure motor stroke - hemiparesis, hemiplegia (+/- dysarthria, dysphagia, transient sensory symptoms)
Pure sensory stroke - persistent/transient numbness, tingling, pain, burning sensations
Dysarthria/clumsy hand - dysarthria and clumsiness
Ataxic hemiparesis - cerebellar and motor symptoms (weakness, clumsiness)
410
What is ESR?
Erythrocyte sedimentation rate - rate at which RBCs sediment in a period of one hour. Non-specific measure of inflammation.
411
What is small artery lipohyalinosis?
Small artery wall vessel thickening, resulting in a smaller luminal diameter.
412
What causes small artery lipohyalinosis?
It is the result of hypertension.
413
What complications are associated with small artery lipohyalinosis?
Can cause small aneurysms or hypertrophy of BVs causing clots or lacunar strokes.
414
What is transient ischaemic attack (TIA)?
Warning 'mini stroke' where the symptoms resolve within 24 hours. Most resolve within 1-60 mins. 10% recurrence within first 2 weeks.
Due to reversible ischaemia and tissue is still viable.
415
What causes a TIA?
Temporary obstruction of blood flow to part of the brain.
416
What symptoms are associated with a TIA?
Same sudden symptoms as stroke (limb weakness, numbness, visual disturbance, speech problems). BUT symptoms last for LESS than 24h.
417
How do you treat patients with a TIA?
Medications to prevent stroke (anti-platelets, anti-hypertensives, statins, endarectomy (if symptomatic ICA stenosis)).
418
What are the symptoms of an ACA occlusion?
Contralateral paralysis of foot and leg, sensory loss over foot and leg, impairment of gait and stance.
419
What symptoms/signs are associated with MCA occlusion?
Contralateral paralysis of face/arm/leg, sensory loss face/arm/leg, homonymous hemianopia, gaze paralysis to opposite side, aphasia (if dominant (left) side), unilateral neglect and agnosia for half external space if non-dominant (right) lobe.
Remember - gaze deviation away from paralysis but towards side of stroke.
420
What parts of the brain are affected in POCS stroke?
Brainstem, cerebellum, thalamus, hypothalamus, occipital lobes.
421
If temporal lobe is affected what can this cause?
Memory problems.
422
What are the symptoms associated with brainstem dysfunction?
Coma, vertigo, nausea, vomiting, cranial nerve palsy, hemiparesis, hemisensory loss, ataxia, crossed sensori-motor deficits, visual field deficits.
423
What symptoms/signs are associated with damage to the right hemisphere?
Left hemiplegia, homonymous hemianopia, neglect syndromes (agnosias).
424
What are the various types of agnosias?
Visual agnosia
Sensory agnosia
Anoagnosia - denial of hemiplegia
Prosopagnosia - failure to recognise faces
425
What is the pathophysiology of Parkinson's syndrome?
Degeneration of neurones in substantia nigra and their dopaminergic (excitatory) inputs into the striatum.
Dopamine can enhance cortical inputs through direct pathway and surpasses inputs through indirect pathway. Depletion of dopamine closes down activation of the focussed motor activities that funnel through thalamus to SMA.
426
What signs/symptoms are associated with Parkinson's disease?
Signs - tremor of hand and jaw and rigidity (increased muscle tone).
Symptoms - hypokinesia (slowness, difficulty making voluntary movements).
427
What signs/symptoms are associated with Huntington's syndrome?
Signs - characteristic chorea (spontaneous, uncontrolled rapid flocks and major movements with no real purpose).
Symptoms - hyperkinesia with dementia and personality disorders.
428
What is Huntington's syndrome?
Inherited condition that causes damage to certain nerve cells in the brain. It is progressive and fatal.
429
What causes the symptoms/signs of Huntington's?
Profound loss of caudate, putamen, globes pallid us (loss of ongoing inhibitory effects of basal ganglia). Can also be accompanied by cortical loss as the striatum is reduced.
430
What are the causes of sudden painless loss of vision?
```
Central retinal vein occlusion
Central retinal artery occlusion
Ischaemic optic neuropathy
Giant cell arteritis
Optic neuritis
Stroke
Vitreous haemorrhage
Retinal detachment
Sudden discovery of pre-existing unilateral LoV
```
431
What happens in central retinal vein occlusion?
Sudden, painless loss of vision.
| Blockage of retinal veins - causing blood/fluid to leak over back of retina, obscuring vision.
432
What will you see when you investigate central retinal vein occlusion?
Haemorrhage and fluid in centre part of retina, dome instead of dip in fovea.
433
What can cause central retinal vein occlusion?
Hypertension, glaucoma, hyperviscosity, inflammation, usually due to a blood clot.
434
What does the retina look like in central retinal artery occlusion?
Pale as no blood getting to it, will see cherry red spot, NO BLOOD.
435
What causes central retinal artery occlusion?
Emboli (carotids/heart), inflammation.
436
What happens in ischaemic optic neuropathy?
Loss of vision due to damage to the optic nerve as a result of ischaemia.
437
What are the two types of ischaemic optic neuropathy? In which is prognosis best?
1. Arteritic (AION) - artery which supplies the optic nerve is affected (mostly commonly due to giant cell arteritis)
2. Non-arteritic (NAION)
NAION (in AION vision will never come back and will lose vision in affected eye).
438
What are the signs of ischaemic optic neuropathy?
Optic disc oedema, afferent pupillary defect.
439
What are the causes of gradual painless loss of vision?
```
Cataract
Refractive Error
Age related macular degeneration
Open angle glaucoma
Diabetic retinopathy
Hypertensive retinopathy
Inherited retinal dystrophies
Drug-induced retinopathy
Other acquired maculopathies
```
440
What are the two types of age related macular degeneration?
```
Wet type (80-90%) - presents with blood and fluid in centre of eye, popping out of fovea, happens suddenly
Dry type (10-20%) - presents with atrophy of macula and Drusen and photoreceptor atrophy.
```
441
What is Drusen?
Lipid deposition in part of the eye.
442
What symptoms are experienced in age related macular degeneration?
Progressive loss of central vision.
443
What are the risk factors associated with age related macular degeneration?
Age, smoking, poor diet.
444
What is the management for age related macular degeneration?
No treatment for dry type.
445
What happens in diabetic retinopathy?
Diabetes leads to microaneurysms in capillary of eyes and capillary fallout. If affects retinal capillaries can lead to ischaemic areas of retina creating black spots in vision.
New BVs may form as a result of widespread ischaemia of the retina - further obscuring vision. After these BVs form, membranes can form around them (membranes + BVs can cause retinal detachment (advanced disease)).
446
What signs are associated with diabetic retinopathy?
Cotton wool spots of ischaemic retina and fluid leaks into eye on fundoscopy.
447
How is diabetic retinopathy managed?
Injection to reduce fluid. Don't tend to treat unless new BV formation. Laser surgery can help get rid of new BVs. Scarring go retina prevents formation of new BVs and gets rid of old ones.
448
What is maculopathy?
Fluid or blood leaking into the posterior part of eye can lead to macula being affected and vision will be really affected if this is the case.
449
What happens in hypertensive retinopathy?
Arteries are not supplying the retina properly, leading to ischaemia. Normally doesn't cause formation of new BVs.
450
What are inherited retinal dystrophies?
Group of inherited conditions affecting photoreceptor function leading to progressive vision loss.
451
What is retinitis pigmentosa? What are the different types of inheritance?
Predominant rods affected leading to night blindness, will see retinal pigmentation (black spots), thin BVs and a pale optic disc.
Peripheral visual loss progressing to central vision loss.
Sporadic, dominant, recessive, X linked and other.
452
What are cone dystrophies? Give example of one.
Mostly sporadic. Phototopic ERG reduced scotopic normal, e.g. Leber's congenital amaurosis.
453
What is Best's vitelliform macular dystrophy?
A RPA-dystrophy (EOG reduced). Usually forms a scar on the retina, vision lost as it involves the macula. Inheritance is dominant.
454
What are the other types of RPA-dystrophies?
Stargardt macular dystrophy (recessive), Sorsby macular dystrophy (dominant), north Carolina macular dystrophy (dominant).
455
What is choroidaemia?
X-linked recessive. Dystrophy of choroid layer. If it becomes damaged, retina becomes pale due to lack of blood supply and BVs swell.
456
What are two types of vitreoretinal dystrophies?
Stickler syndrome
| Congenital retinoschisis
457
How do you investigate muscle disease?
History and examination
Creatinine phosphokinase (raised in some degenerative muscle dx)
Electrical myography (needle in muscle and see how it responds electrically)
Muscle biopsy (structure, biochemistry (enzyme deficiency?), inflammation
Genetic testing
458
What are the different classifications of muscle disease? Give a brief description of each of the 6 types.
Muscular dystrophies - often genetic, something wrong with structure of muscle. Usually presents in 30s. Muscle degeneration leading to weakness. Not do with NMJ.
Channelopathies - as you need sodium, chlorine and calcium to make muscle contract problems with these channels can't present with episodic weakness or stiff muscles that won't relax.
Metabolic muscle disease - defect in energy usage in muscle, usually problem with enzyme.
Inflammatory muscle disease - inflammation.
Congenital myopathies - born with.
Iatrogenic - medications.
459
Which drug can cause inflammation/degeneration of muscle?
Statins, patients present with muscle pain and weakness.
460
What conditions may patients be predisposed to as a result of having a muscular disease?
Cardiomyopathies, arrhythmias, respiratory problems.
461
What are the different types of muscular dystrophies?
Duchenne's MD, Becker's MD, Facioscaphulohumeral MD, Myotonic dystrophy, Limb-girdle MD.
462
Duchenne's MD
- aetiology
- inheritance
- signs/symptoms
- prognosis
- Genetic mutation leading to absence of dystrophin.
- Affects boys in early childhood. X-linked recessive gene. 1/3 de novo.
- Hypertrophied, weak calves (fatty infiltration), high CK, progressive muscle degeneration and weakness.
- Live to 20s.
463
Becker's MD
- aetiology
- inheritance
- signs/symptoms
- prognosis
- Milder form of Duchenne's. Mutations in dystrophin gene leading to misshapen dystrophin.
- Develops in later childhood. X-linked recessive.
- Hypertrophied, weak calves (fatty infiltration), elevated CK, muscle weakness, skeletal deformities.
- Longer survival that Duchenne's.
464
Facioscapulohumeral
- aetiology
- inheritance
- signs/symptoms
- prognosis
- Autosomal dom inherited condition.
- Develops in childhood/adulthood.
- Initially affecting skeletal muscles of face, scapula and shoulders (weakness).
- Progresses slowly. Not life-threatening usually.
465
Myotonic Dystrophy
- inheritance
- symptoms
- prognosis
- Any age, autosomal dom.
- Muscle loss/weakness.
- Life expectancy only affected if severe form.
466
Limb-girdle MD
- inheritance
- symptoms
- prognosis
Note this is a group of conditions.
- Develops in late childhood/early adolescence, autosomal dom.
- Progressive muscle wasting affecting mostly hip and shoulder muscles.
- Variable life expectancy.
467
What is dystrophin? What happens to it in Becker's/Duchenne's.
Rod shaped protein that connects the cytoskeleton of the muscle fibre to the surrounding extracellular matrix through the cell membrane. Lack of this protein leads to a floppy/broken sarcolemma in which CK can escape and calcium can enter, ultimately leading to muscle death.
468
Channelopathies predominantly affect which channels?
Sodium, calcium and chloride.
469
Whata re the 4 types of channelopathies?
Familial hypokalemic periodic paralysis
Hyperkalemic periodic paralysis
Paramytonia congenita
Myotonia congenita
470
What is familial hypokalemic periodic paralysis?
Rare, autosomal dom condition that causes occasional episodes of muscle weakness that lasts mins-days.
471
What is hyperkalemic periodic paralysis?
Autosomal dominant disorder of sodium channels (also unable to regulate plasma potassium --> increase potassium in the blood). Causes episodes of extreme muscle weakness/paralysis.
472
What is paramyotonia congenita?
Autosomal dominant condition that causes paradoxical myotonia (becomes worse with exercise, whereas classical myotonia improves).
473
What is myotonia?
Delayed relaxation of a muscle.
474
What is myotonia congenita?
Failure of initiated contraction to terminate and rigidity. Repeated contraction of muscle alleviates stiffness.
475
What are the various types of metabolic muscle disease? Give an example of each.
Disorders of carbohydrate metabolism - e.g. problem with glycogen breakdown (presenting on intense exercise).
Disorders of lipid metabolism - will present on prolonged exercise.
Mitochondrial myopathies/cytopathies - defects in mitochondria leading to prominent muscular symptoms. Tend to be genetic. Muscle weakness and exercise intolerance common symptoms.
Endocrinopathy - e.g. Cushing's, thyroid disease.
Biochemical abnormalities - e.g. prolonged vomiting --> low K and patient can experience profound muscle weakness.
476
How do you investigate Cushing's syndrome?
Blood, urine, saliva to measure cortisol levels.
477
What causes Cushing's syndrome?
Very high levels of cortisol. Can be due to cortisol treatment, inflammation or autoimmune conditions.
478
What are the signs/symptoms of Cushing's syndrome?
Fat deposits in face, red-purple striae, weight gain, thinning of skin that bruises easily, muscle/bone weakness, loss of libido.
479
How is Cushing's syndrome managed?
Decrease/withdraw corticosteroid use. CAREFULLY to prevent Addison's.
480
What are the two types of inflammatory muscle disease?
Polymyositis and dermatomyositis.
481
What is polymyositis?
Condition causing inflammation of ONLY the muscle. Often autoimmune (30-50s mostly affected). Presentation can be subacute/acute with painful weak muscles on both sides and systemically unwell.
482
How do you investigate polymyositis?
CK (raised), EMG, biopsy (inflammation and myopathy), CD8 cells may be found in bloods.
483
What is dermatomyositis?
Inflammation of muscle AND skin. Can be autoimmune or paraneoplastic (40-60s mostly affected). Presents with subacute/acute painful, weak muscles, systemically unwell and with characteristic rash.
484
How do you investigate dermatomyositis?
CK (raised), EMG, biopsy (inflammation and myopathy), humeral mediated B cells and CD4 cells.
485
How do you treat polymyositis and dermatomyositis?
Immunosuppression (steroids/steroid-sparing).
486
What is the aetiology involved in myasthenia gravis?
Disorder affecting all NMJs apart from heart. Patient produces antibodies against ACh receptor. Will have surplus of ACh in morning which can flood ACh receptors so Ab won't bind as much, but as it is used up, Ab starts to block receptor --> fatiguable weakness.
487
How can you investigate fatiguable weakness to determine if a patient has myasthenia gravis?
AChR Ab, anti-MuSK Ab (associated with receptors and needed for receptor to work properly), neurophysiology (repetitive stimulation of muscle to see fatigue), will see jitter on EMG or block in really severe cases), do CT chest (thymoma can be associated with MG).
488
What symptoms are associated with myasthenia gravis?
Fatiguable weakness of limbs, eyelids (ptosis), muscle of mastication (chewing/swallowing), talking, SoB, diplopia.
489
How is myasthenia gravis treated?
Symptomatic Rx: acetylcholinesterase inhibitor (blocks ACh breakdown), immunosuppression (prednisone or steroid sparing (e.g. azathioprine) to block Ab production), immunoglobulin/plasma exchange (acute situations for temporary relief), thymectomy (if overactive thyroid).
490
What are the different types of nerve disease?
Root disease
Lesion of individual peripheral nerve
Generalised peripheral neuropathy
491
What signs are associated with root disease?
Myotomal wasting and weakness, reflex change, dermatomal sensory change.
492
What signs are associated with lesion of individual peripheral nerves?
Wasting and weakness of innervated muscle, specific sensory change.
493
What symptoms are associated with generalised peripheral neuropathy?
Sensory and motor symptoms, starting distally and moving proximally.
494
What can cause root disease?
Degenerative spine disease, inflammation or infiltration.
495
What can cause lesion of individual peripheral nerves?
Compressive/entrapment, e.g. Carpal Tunnel Syndrome
| Vasculitis (e.g. Mononeuritis Multiplex)
496
What nerves are generally affected first in generalised peripheral neuropathy?
The longest nerves (as they require the most energy to move everything down one axon), presentation therefore is in hands and feet first.
Can also get autonomic features.
497
How do you investigate nerve disease?
Blood tests (B12, diabetes, alcohol), genetic analysis, nerve conduction studies, LP (CSF analysis), nerve biopsy.
498
What is carpal tunnel syndrome?
Compression of the medial nerve as it runs through the wrist, leading to pain, numbness and tingling in thumb, index and middle finger.
499
How is carpal tunnel syndrome managed?
Surgery.
500
What can cause generalised peripheral neuropathy?
Metabolic causes - diabetes, alcohol (cerebellar damage --> ataxia, dementia, peripheral neuropathy), B12 deficiency, renal problems.
Toxic, e.g. drugs
Hereditary
Infection, e.g. Lyme disease, HIV, leprosy
Malignancy - paraneoplastic syndromes
Inflammatory demyelination - Guillan Barre syndrome, chronic inflammatory demyelination polyneuropathy.
501
What can cause Guillain Barre Syndrome?
Autoimmune dsiease, triggered possible my infection (campylobacter). Proteins of this bacteria v. similar to myelin sheath proteins so immune system gets confused and starts destroying its own myelin.
502
What symptoms result from Guillain Barre Syndrome?
Numbness/weakness/pain of feet, hands and limbs.
503
What is the treatment for Guillain Barre Syndrome?
IV immunoglobulin, plasmapheresis, may require ventilation.
504
What must you be mindful of in Guillain Barre Syndrome?
Patient may need respiratory support.
505
What is the prognosis for Guillain Barre Syndrome?
Most make a fully recovery, but can take months-years to recover.
506
What are UMN signs?
Increased tone, brisk (increased) reflexes, no sensory involvement, pyramidal pattern of muscle weakness tend to get no muscle wasting. Babinski positive.
May get muscle wasting in late disease, but more likely due to muscle disease.
507
What are LMN signs?
Muscle fasciculations, wasting, weakness.
508
Patient presents with pericroneal redness with mild pain and no discharge, vision is slightly blurred - what is the diagnosis?
Uveitis.
509
Patient presents with peripheral diffuse redness with itch sensation and yellow discharge, vision is unaffected - what is the diagnosis?
Bacterial conjunctivitis.
510
Patient presents with peripheral diffuse redness with itch sensation and watery discharge, vision is unaffected - what is the diagnosis?
Viral conjunctivitis.
511
Patient presents with peripheral diffuse redness with itch sensation and mucous discharge, vision is unaffected - what is the diagnosis?
Allergic conjunctivitis.
512
Patient presents with sectorial/diffuse redness with severe pain and no discharge, vision is unaffected - what is the diagnosis?
Scleritis.
513
Patient presents with pericorneal redness with severe eye pain, headache and discharge, vision is lost - what is the diagnosis?
Acute glaucoma.
514
Patient presents with pericorneal redness with severe pain/eye closing and discharge, vision is lost - what is the diagnosis?
Keratitis/corneal ulcer.
515
Patient presents with severe periocular pain and no discharge, vision is unaffected - what is the diagnosis?
Orbital cellulitis.
516
What is episcleritis?
Inflammation of the superficial, episcleral layer.
517
What is scleritis?
Inflammation of the sclera.
518
What symptoms are associated with episcleritis and scleritis?
Pain and redness and NO vision loss.
519
How are episcleritis and scleritis treated?
Scleritis always requires systemic treatment. Episcleritis is self-limiting.
520
What are the possible complications associated with scleritis?
Severe ocular inflammation, often with ocular complications.
521
What is necrotising scleritis?
Inflammation of the sclera, looks white, difficult to treat. Sclera thins out and can lead to perforation. Sometimes have to do scleral patch.
522
What is a tumour?
Abnormal proliferation of tissue.
523
What is a benign tumour?
Normal cells in abnormal numbers/locations. Lack ability to invade local tissue/metastasise, slowly growing. BUT must consider mass effect.
524
What is a malignant tumour?
Anaplastic cells (loss of form/function), often growing rapidly, capable of invading surrounding tissue and spreading to distant locations.
525
What is a carcinoma?
Malignant tumour derived from epithelial cells.
526
What is a sacroma?
Malignant tumour derived from connective tissue.
527
What is a lymphoma?
Malignant tumour derived from lymphatic tissue.
528
What is a leukaemia?
Malignant tumour derived from blood tissue.
529
What is a blastoma?
Malignant tumour derived from immature precursor cells or embryonic cells.
530
What is a big risk factor for cerebral venous thrombosis?
OCP.
531
What can cause a cerebral venous sinus thrombosis?
Infection spreading from mastoids or orbit, inherited/acquired thrombophilic states (e.g. OCP, dehydration, cancer, sepsis, myeloproliferative disorders, pregnancy, puerperium.
532
What symptoms are associated with cerebral venous sinus thrombosis?
Headache, with stroke, seizures, decreased consciousness, intracranial hypertension can cause nausea, vomiting, papilloedema and visual obscuration.
533
How is a cerebral venous sinus thrombosis treated?
Anti-coagulation, even if there is a haemorrhagic venous infarct.
534
What is meningitis?
Inflammation of the meninges.
535
What investigations can you carry out if you suspect meningitis?
Nick stiffness tested for by passive bending of neck backwards, blood culture (bacteraemia), LP (CSF microscopy/culture), no need for imaging if no CIs to LP.
536
In which condition will you see petechial skin rash?
Meningiococcal meningitis, may also occur in viral meningitis.
Can use tumblar test (to test for a non-blanching rash).
537
What are the CSF findings in bacterial meningitis?
Increased opening pressure, high cell count (mainly neutrophils), reduced glucose, and high protein content.
538
What are the CSF findings in viral meningitis and encephalitis?
Normal or increased opening pressure, high cell count (mainly lymphocytes), normal glucose and slightly increased protein.
539
If bacterial meningitis is suspected what Ix must you carry out?
Blood culture gram stain (gram positive cocci in chains - streptococcus, if you get this - culture to see if sensitive to penicillin).
540
What are the differentials for meningitis?
Infective - bacteria (Neisseria meningitidies (meningococcus), streptococcus pneumonia (pneumococcus), viral (incl. enteroviruses), fungal.
Inflammatory (sarcoidosis)
Drug induced (NSAIDs, IVIG)
Malignant (metastatic, haematological, e.g. leukaemia, lymphoma, myeloma).
541
What are the classic triad of symptoms in meningitis? And what other symptoms can patients present with?
Fever, neck stiffness and altered mental status (confusion, delirium, declining conscious level is common (GCS <14)).
Headache, nausea, vomiting, cranial nerve palsy and focal neurological deficits can also be present.
542
How is meningitis treated?
Antibiotics, fluids IV, oxygens.
543
How can meningitis be prevented?
Vaccination available against meningitis causing agents.
544
What are the possible complications of meningitis?
Septicaemia.
545
What is encephalitis?
Inflammation/infection of the brain substance.
546
How can encephalitis be investigated?
Blood cultures, imaging (CT, MRI), LP, EEG, test for HSV encephalitis (lab diagnosis by PCR of CSF for viral DNA).
547
What are the differentials for encephalitis?
Infective - viral (most common HSV)
Inflammatory - limbic encephalitis (anti-VGKC, anti-NMDA receptor, ADEM)
Metabolic - hepatic, uraemia, hyperglycaemic
Malignant - metastatic, paraneoplastic
Migraine, post ictal
548
What symptoms are associated with encephalitis?
Flu-like produce (4-10days), progressive headache w/ fever, +/- meningism, progressive cerebral dysfunction (confusion, abnormal behaviour, memory disturbance, depressed conscious level), seizures, focal signs/symptoms.
Onset viral encephalitis slower than bacterial meningitis and cerebral dysfunction more prominent.
549
How is encephalitis treated?
Aciclovir on suspicion of HSV encephalitis.
550
What antibodies are involved in autoimmune encephalitis?
Two important ones -
- anti-VGKG (voltage gated potassium channel)
- anti-NMDA receptor
551
Is autoimmune or viral encephalitis more common?
As common as one another.
552
What symptoms are associated with the two main types of autoimmune encephalitis?
Anti-VGKG - frequent seizures, amnesia, altered mental state.
Anti-NMDA - flu-like produce, prominent psychiatric features, altered mental state, progressive to a movement disorder and coma.
553
What causes arbovirus encephalitidies?
Arthropob borne virus group. Commoner in other parts of the world - travel history essential.
554
What vectors are involved in the spread of arbovirus encephalitidies?
E.g. mosquito or tick from non-human host (e.g. West Nile virus, St Louis Encephalitis)
555
How is arbovirus encephalitidies prevented?
Some prevented by immunisation.
556
What are the CIs to LP?
Focal neurological deficit, not incl. cranial nerve palsies
New onset seizures
Papilloedema
Abnormal level of consciousness, interfering with proper neurological examination (GCS<10 suggests raised ICP)
Severe immunocompromised state
Focal symptoms or signs suggesting focal brain mass
557
What viruses are included in the herpes group of viruses?
VZV, EBV, CMV, HSV 1 and 2.
558
Which of the herpes simplex viruses causes cold sores and which causes genital herpes?
1 - causes cold sores more than type 2.
| 1 and 2 both cause genital herpes.
559
How do you cure herpes?
Virus remains latent in trigeminal or sacral ganglion after primary infection. Therefore there is no cure.
560
What is a rare complication of HSV?
Encephalitis (other than neonates all caused by type 1).
561
What are enteroviruses?
Tend to cause CNS (neurotopic) infections in humans, no animal reservoir.
Spread by faecal-oral.
May cause non-paralytic meningitis. DO NOT cause gastroenteritis.
E.g.s - poliovirus, coxsackieviruses and echoviruses.
562
What is a brain abscess?
Localised area of pus within the brain.
563
What is a subdural empyema?
Thin layer of pus between the dura and arachnoid membranes over the surface of the brain.
564
How do you investigate a brain abscess or empyema?
CT/MRI, blood culture, biopsy.
565
What are the common organisms that cause brain abscesses or empyemas?
Streptococcus in 70% (esp in penicillin sensitive 'strep miller' group (strep anginosus, strep intermedium, strep constellates), anaerobes in 40-100% of cases (bacteroides/prevotella).
566
What can cause brain abscesses or empyemas?
Dental, sinus or ear infections, penetrating head injuries, blood borne infections (e.g. bacterial endocarditis), neurosurgery).
567
What are the signs of brain abscesses or empyemas?
Raised ICP signs (papilloedema, false localising signs, depressed conscious level), meningism (more in empyema).
568
What symptoms are associated with brain abscesses or empyemas?
Fever, headache, focal symptoms/signs (dysphagia, seizures, hemiparesis).
569
How do you treat a brain abscess?
Surgical drainage if possible penicillin or ceftriazone to cover streps, metronidazole for anaerobes - HIGH DOSES.
570
What are HIV indicator illnesses in the brain?
```
Cerebral toxoplasmosis
Aseptic meningitis/encephalitis
Primary cerebral lymphoma
Cerebral abscess
Cryptococcal meningitis
Space occupying lesion of unknown cause
Dementia
Leukoencephalopathy
```
571
What sort of brain infections will you tend to see in patients with HIV and low CD4 counts?
Cryptococcus neoformans
Toxoplasma gondii
Progressive multifocal leukencephalopathy (PML)
Cytomegalovirus (CMV)
HIV-encephaloapthy (HIV-associated dementia)
572
What is india ink used to stain?
Crytococcus neoformans.
573
Cryptococcuseoformans has a worldwide distribution in what?
Soil and avian habitats.
574
Cryptococcus gatti is associated with what?
Trees.
575
How do you investigate cryptococcal infections?
Mucicarmine-stained histological sections (may show abundance of pink-stained fungi in AIDs patients with cryptococcal menigioencephalitis)
India ink can be used to stain cytococcus neoformans.
576
What are risk factors for cryptococcal infections?
Inhalation of airborne organisms, immunosuppression (AIDs, immunosuppressive drugs (particularly in solid organ transplant)). C. gatti infection can occur in immunocompetent but this is rare.
577
What are the symptoms associated with cryptococcal infections?
Meningioencephalitis (fever, headache, change in mental status, may present as febrile illness (SoB, coughing, fever), skin lesions.
578
What spirochaete causes Lyme disease?
Borrelia burgorferia (carried by ticks).
579
What spirochaete causes syphillis?
Treponema pallidium.
580
What spirochaete causes leptospirosis?
Leptospira interrogans (spread by infected animal pee).
581
How do you investigate Lyme disease?
Serological tests, CSF lymphocutosis, PCR of CSF, MRI brain/spine, nerve conduction studies/EMG (if PNS involvement).
582
What happens in the first 30 days of infection of Lyme Disease?
Early localised infection.
Characteristic expanding rash (target board - erythema migrans), febrile illness, fatigue, myalgia, arthralgia, headache, fever, chills, neck stiffness.
583
What happens after weeks/months of infection of Lyme disease?
1+ organ systems become involved (haematological/lymphatic spread), MSK and neurological involvement most common).
In untreated patients: mononeuropathy, mononeuritis multiplexus, radiculoneuropathy, cranial neuropathy, myelitis, meningococcus-encephalitis (PNS>CNS).
584
What happens months or years after infection of Lyme disease?
MSK and neurological involvement - subacute encephalopathy, encephalomyelitis most common. DOES NOT cause chronic fatigue syndrome.
585
What systems can be involved in Lyme disease?
Skin, rheumatological, neurological/neuropsyhiatric, cardiac, ophthalmological.
586
How do you treat Lyme disease?
IV cefriaxone and oral doxycycline.
587
How do you investigate syphilis?
Treponema specific and non-treponemal specific (VDRL) antibody tests, CSF lymphocytes increased, evidence of intrathecal antibody production, PCR.
588
What are the risk factors for developing syphilis?
Being in contact with a chancre (sore) or having sex with an infected person/
589
What are three stages of syphilis?
Primary - sores which may heal after 3-6wks.
Secondary - rough, red/brown rash on palms of hands/feet, swollen lymph nodes, fever, sore throat, patchy hair loss, joint aches, fatigue.
Latent/tertiary - neurosyphilis comes year/decades after primary dx.
590
How is syphilis treated?
High dose penicillin.
591
What is leptospirosis? What are the red flags?
Aka. Weil's disease, causes fever, headache, nausea, vomiting, myalgia, red eyes, anorexia.
Red flags - jaundice, oedema of ankles/feet/hands, chest pain, SoB, haematemesis.
592
Name 4 diseases preventable by immunisation.
Poliomyelitis
Rabies
Tetanus
Botulism
593
What is poliomyelitis?
Highly infectious viral disease. Wild polio eliminated in the Uk.
594
What causes poliomyelitis?
Polioviruses 1/2/3 (all enteroviruses) infecting anterior horn cells of lower motor neurone).
595
What symptoms are associated with poliomyelitis?
99% asymptomatic. Paralytic disease in 1% (flaccid paralysis in legs, no sensory features).
Initial symptoms: fever, headache, fatigue, vomiting, stiffness of neck and sore muscles.
596
How is poliomyelitis cured?
There is no cure.
597
What is rabies?
Acute infectious disease of CNS affecting most animals. It is a neurotic virus (enters peripheral nerves and migrates to CNS).
598
How is rabies investigated?
No useful diagnostic tests before clinical disease apparent.
Diagnosis based on culture, detection or serology.
599
How is rabies transmitted to humans?
Bite or salivary contamination of open lesion.
600
What are the risk factors for contracting rabies?
Bat handling (bats recognised to carry rabies like virus (European Bat Lyssavirus 2 (EBL2)).
601
What symptoms are associated with rabies?
Paraesthesia at site of original lesion, ascending paralysis and encephalitis, fever, headache, anxiety, confusion, aggressive behaviour, hallucinations, frothing at mouth, muscle spasms and difficult breathing/paralysis.
602
How do you treat rabies?
Wash wound, give active rabies immunisation, give human rabies immunoglobulin if high risk.
Post exposure Rx 100% effective if started before onset of symptoms.
603
What is tetanus?
Condition caused by infection with clostridium tetani (anaerobic gram +ve bacillus, spore forming). Produces toxin that acts at NMJ and blocks inhibition of motor neurone causing rigidity and asepsis (rises sardonic).
604
What are the risk factors for contracting tetanus?
Infection of cuts, burns, animal bites, eye injuries, body piercings, tattoos, injections. Not spread person to person.
605
What are the symptoms of tetanus?
Rigidity, painful spasms (e.g. jaw lack, fever, sweating, tachycardia).
606
How is tetanus treated?
Penicillin and immunoglobulin for high risk wounds/patients.
607
What causes botulism?
Clostridium botulinum is a neurotoxin that binds irreversibly to presynaptic membranes of peripheral NMJs and autonomic nerve junctions and blocks acetylcholine release.
Body tries to recover by spouting new axons.
608
How is botulism investigated?
Nerve conduction studies, mouse neutralisation bioassay for toxin in blood, culture from derided wound.
609
What sort of bacteria is clostridium botulinum?
Anaerobic spore producing gram +ve bacillus. Naturally present in soil, dust and aquatic environments.
610
What are the three modes of infection in botulism?
Infantile (intestinal colonisation)
Food-borne (outbreaks)
Wound (IVDA/popping drug users).
611
What are the signs/symptoms of botulism?
Autonomic dysfunction (pupil dilatation), incubation period (1-14 days), descending symmetrical flaccid paralysis, pure motor respiratory failure.
612
How is botulism treated?
Anti-toxin (A, B and E), penicillin/metronidazole (prolonged), radical wound debridement.
613
What is the pathophysiology behind post-infective inflammatory syndromes?
Molcular mimicry leading to autoimmune response. Latent interval between precipitating infection and onset of neurological symptoms.
614
Give an example of a post-infective inflammatory syndrome in the CNS?
Acute disseminated encephalomyelitis (ADEM).
615
Give an example of a post-infective inflammatory syndrome in the PNS?
Guillian Barre Syndrome (GBS).
616
What is acute disseminated encephalomyelitis?
Autoimmune disease marked by sudden, widespread attack and inflammation of the brain and spinal cord. Can cause damage to the myelin sheaths of CNS.
617
What can trigger acute disseminated encephalomyelitis?
Viral infection or non-routine vaccinations.
618
What are the symptoms of acute disseminated encephalopmyelitis?
Resembles MS, but single flare up.
Major symptoms incl. fever, headache, vomiting, confusion, vision impairment, drowsiness, coma, seizures.
619
How do you treat acute disseminated encephalopmyelitis?
No specific Rx, corticosteroids to reduce inflammation.
620
What is creutzfeldt-Jacob disease?
CJD appears to be caused by an abnormal infectious protein called a prion. These prions tend to accumulate at high levels in the brain and cause irreversible damage to brain cells.
621
What are the different types of CJD?
Sporadic CJD
New variant CJD
Familial CJD
Acquired CJD (cadaveric growth hormone, dura mater grafts, blood transfusion).
622
What must you consider as a differential for any rapidly progressing dementia?
Sporadic CJD.
623
What is the most common type of CJD?
Sporadic.
624
What is the aetiology behind sporadic CJD?
Believed to be abnormal brain proteins misfolding abnormally and turning into a prion.
625
What are the differentials for sporadic CJD?
Alzheimer's disease with myoclonus, subacute sclerosing pancephalitis (SSPE - v. rare, chronic infection with defective measles virus), CNS vasculitis, inflammatory encephalopathies.
626
What signs/symptoms are associated with sporadic CJD?
Insidious onset (>60s usually), early behavioural abnormalities, rapidly progressive dementia, myoclonus --> global neurological decline, motor abnormalities (cerebellar ataxia, extrapyramidal (tremor, rigidity, bradykinesis, dystonia), pyramidal (weakness, spasticity, hyperreflexia), cortical blindness, seizures.
627
What is the prognosis in sporadic CJD?
Death often in 6mths.
628
What signs can you see in new variant CJD?
Pulvinar sign on MRI
EEG - generalised period complexes typical
CSF - normal or raised protein immunoassay 14-3-3 brain protein
629
What can cause new variant CJD?
Consumption of meat from cow with bovine spongiform encephalopathy (mad cow disease). Similar prion to CJD can also be spread by blood transfusion. May be genetic susceptibility.
630
How do the signs and symptoms of new variant CJD vary in comparison to sporadic CJD?
Early behavioural changes more prominent and longer course (avg 13 months).
631
What is pulvinar's sign?
Seen in new variant CJD - refers to bilateral flair hypersensitivies involving the pulvinar thalamic nuclei.
632
What is post-traumatic amnesia?
Period of recovery following traumatic brain injury whereby the patient experiences memory problems, these patients are disorientated (unable to locate themselves in time and place).
633
What are the two types of amnesia and how do they differ?
Anterograde amnesia - inability to remember new events/experiences after injury (due to hypothalamic damage).
Retrograde amnesia is the loss of memories that were formed shortly before injury. This is due to damage to the thalamus
634
What is a coma?
A state of unarousable psychological unresponsiveness in which subjects lie with their eyes closed and show no psychologically understandable response to outside stimuli.
635
How do you investigate someone in a coma?
Temperature, HR, BP, CVS, respiration, skin, breath, abdomen, meningism, fundal examination, CGS, brainstem function, motor function, reflexes.
636
What are causes of a low GCS?
Toxic/metabolic states (hypoxia/hypercapnia/sepsis/hypotension/drug intoxication/renal/liver failure/hypoglycaemia/ketoacidosis)
Seizures
Damage to reticular activating system
Causes of raised ICP (tumour, stroke, EDH, SDH, SAH, hydrocephalus)
637
Write down the Glasgow Coma Score.
EYE OPENING
4. spontaneous
3. to speech
2. to pain
1. none
BEST VERBAL RESPONSE
5. orientated
4. confused
3. inappropriate words
2. incomprehensible
1. none
BEST MOTOR RESPONSE
6. obeying commands
5. localising to pain
4. withdrawing from pain
3. flexing to pain
2. extending to pain
1. none
638
What signs/symptoms and GCS relate to being in a coma?
Failure to open eyes in response to voice, perform no better than weak flexion in response to brain and are incomprehensible.
GCS of 8 or less.
639
What is important to remember when using GCS?
Motor function dependent on motor response, muscle tone, tendon reflexes, seizing.
640
How do you manage someone in a coma?
Maintain vital functions, care for skin (avoid pressure sores), attention to bladder/bowel function, control seizures, prophylaxis of DVT, peptic ulcerations, contractors. CONSIDER locked in syndrome.
641
In non-traumatic coma, after how many hours will prognosis rapidly decline if patient cannot be lifted from coma?
6h.
642
If a patient is in a coma, but there is no meningism or focal brainstem/lateralising cerebral signs what investigations can you do and what differentials would you think of?
Ix - toxicology screen (alcohol level, blood sugar, electrolytes), assess hepatic and renal function, acid-base assessment and blood gases, BP, CO poisoning.
Differentials - anoxia/ischaemic conditions, metabolic disturbances, intoxications, systemic infections, hyper/hypothermia, epilepsy.
643
If a patient is in a coma, and there is meningism what are your differentials and what investigations can you carry out to confirm a differential?
Differentials - SAH, meningitis, encephalitis.
Ix - CT head, LP (appearance, cell count, glucose level, capsular antigen tests).
644
If a patient is in a coma, and has focal brainstem or lateralising cerebral signs what investigations could you do and what would your differentials be?
Ix - CT/MRI (if not diagnostic, Ix for other causes of coma, e.g. metabolic screens...)
Differentials - cerebral tumours, cerebral haemorrhage, cerebral infarct, cerebral abscess.
645
What are the medical causes of a coma lasting more than 5h?
Drugs/alcohol
Hypoxia, may be secondary to MI
Cerebrovascular event, i.e. haemorrhage/infarction
Metabolic, e.g. diabetes, heptic failure, renal failure, sepsis, hyerpcapnia/hypoxia.
646
How can you distinguish a subdural haematoma from an extradural haematoma on CT?
Subdural haematoma forms an ellipse (convex), extradural haematoma forms a concave shape (lens).
647
Why can head injury lead to focal neurological signs/epilepsy?
Diffuse axonal injury, contusion, intracerebral haematoma, extra-cerebral haematoma (extradural haematoma, subdural haematoma).
648
How do you manage someone with a head injury?
Stabilise cervical spine, ABC if GCS 8 or less (intubate, ventilate), treat raised ICP, decompressive surgery or removal of haematoma, neuro-observation.
649
What is persistent vegetative state?
A state in which the brainstem recovers to a considerable extent but there is no evidence of recovery of cortical function. Arousal and wakefulness but patient does not regain awareness of purposeful behaviour of any kind.
650
What is locked-in syndrome?
Diagnosis dependen ton recognising patient can open their eyes voluntarily and signal numerically by eye closure.
Patient has total paralysis below level of the third nuclei and although able to open, elevate and depress the eyes, has no horizontal eye movement and no other voluntary movement.
651
What might explain depressed respiration?
OD, metabolic disturbance.
652
What might explain increased respiration?
Hypxoia, hypercapnia, acidosis.
653
What might explain fluctuating respiration?
Brainstem lesion.
654
What is involved in resus?
Airway
Breathing
Circulation
Blood samples - glucose, biochem, haematology, blood gas, toxicology.
Find BP, pulse, temp, IV access, stabilise the neck.
655
What signs and symptoms are associated with a C6 spinal cord lesion?
Increased tone in legs, brisk reflexes, babinski +ve.
Weakness in elbow below sensory level C6.
656
What signs and symptoms are associated with an L4 nerve root lesion?
Numbness in L4 dermatome, weakness in ankle dorsiflexion, reduced knee jerk. Pain down ipsilateral leg.
657
What is a radiculopathy?
Compression of nerve root leading to dermatomal and myotomal deficits. Leads to pain in single dermatome and loss of reflexes.
658
What are the reasons for compression of the spine?
```
Degenerative
Tumour
Infection
Trauma
Congenital
```
659
How do you investigate compression of the spine?
History, exam, bloods, X-rays, CT, MRI.
660
What is disc prolapse?
Acute herniation of IV discs causing compression of spinal roots or spinal cord. Tends to be in younger patients.
661
How do you investigate a disc prolapse?
MRI.
662
What symptoms are associated with disc prolapse?
Acute onset pain down leg/arm, numbness, weakness in distribution of nerve root involved.
663
How is disc prolapse treated?
Rehab, nerve root injection, lumbar/cervical discectomy.
664
Compression of central spine in cervical region leads to what?
Cervical myelopathy.
665
Compression of lateral spine in cervical region leads to what?
Cervical radiculopathy.
666
Compression of central spine in lumbar region leads to what?
Cauda equina syndrome.
667
Compression of lateral spine in lumbar region leads to what?
Lumbar radiculopathy.
668
What is sciatica? What is it caused by?
Irritation of the sciatic nerve (runs from hips to feet). Can cause tingling, pain, numbness, weakness in bottom, legs, get and toes. Can result from slipped disc.
669
What is cauda equina syndrome?
MEDICAL EMERGENCY.
| Symptoms due to damage of cauda equina.
670
What are the red flags for cauda equina syndrome?
Bilateral sciatica, saddle anaesthesia (loss of sensation to area of buttock, perineum, inner thighs), urinary dysfunction.
671
How is cauda equina syndrome investigated?
Clinical-radiological diagnosis. Urgent MRI.
672
How is cauda equina syndrome treated?
Emergency lumbar discectomy.
673
What are degenerative spinal problems?
Loss of normal spinal structure, seen in older patients. Product of disc prolapse, ligamentum hypertrophy, osteophyte formation, leading to myopathies and radiculopathies.
674
What are oestrophytes?
Bone spurs (bony projections) that form along the joint margins of patients with degenerative spinal problems.
675
What is cervical spondylosis?
Umbrella term for degenerative change in cervical spine and nerve root compression. Ageing causes wear and tear to muscles and bones. Common cause of neck pain in over 50s.
676
What symptoms are experienced in cervical spondylosis?
Myelopathy/radiculopathy/both. Develops over months to years (shoulder/neck pain/stiffness that come and go and headaches starting at back of neck).
677
How do you treat cervical spondylosis?
Conservative if no/mild myelopathy, surgery for progressive moderate to severe myelopathy (ant and post approaches).
678
What is lumbar spinal stenosis?
Narrowing of spinal canal in lumbar region, normally due to bone/tissue/both growing out and protruding into the vertebral canals, squeezing and irritating the nerves.
679
What symptoms are experienced in lumbar spinal stenosis?
Pain down both legs (spinal claudication), worse on walking/standing and relieved by sitting/bending forward.
680
How is lumbar spinal stenosis treated?
Lumbar laminectomy.
681
If a cancer patient presents with development of back pain how should you proceed?
Urgent MRI.
682
What are the symptoms of spinal tumours?
Pain, weakness, sphincter disturbance.
683
How do you treat spinal tumours?
Surgical decompression, radiotherapy.
684
What are the different types of spinal tumours? Give a few examples of each.
Intramedullary - astrocytoma, ependymoma, teratoma, haemangioblastoma
Intradural- meningioma, neurofibroma, lipoma
Extradural - mets (lung, breast, prostate), primary bone tumours (chrodomas, osteoblastomas, osteuid oestoma).
685
What is ostomyelitis?
Infection within the vertebral body.
686
What is discitis?
Infection of the interverebtal disc.
687
What is a spinal epidural abscess?
Infection in the epidural space (collection of pus between the dura mater and vertebrae).
688
What are the symptoms of a spinal epidural abscess and how is it investigated?
Triad - backpain, pyrexia, focal neurology.
| Urgent MRI.
689
What organisms most commonly cause a spinal epidural abscess?
Staph aureus, strep, e. coli.
May be post-back surgery, spread from bloodstream, infections, boils on scalp/back or in those with vertebral osteomyelitis.
690
What are the risk factors for spinal epidural abscess?
IVDA, diabetes, chronic enal failure, alcoholism.
691
How do you treat a spinal epidural abscess?
Urgent surgical decompression and long term IV antibx.
692
What are the risk factors for osteomyelitis?
IVDA, diabetes, chronic renal failure, alcoholism, AIDs, recent bone fracture.
693
What symptoms are associated with osteomyelitis?
Pain in long bones of legs most commonly, can also affect bones in back/arms.
694
How do you treat osteomyelitis?
Antibx and surgery if evidence of neurology. Drain abscesses.
695
What are the indications for CT?
Head trauma, acute stroke, headache (red flags for SAH), cancer (brain mets in patients with no symptoms), post-surgical (hydrocephalus, haemorrhage).
696
What are contraindications for CT scans?
GCS 15, no suspected open/depressed skull fracture, no haemotympanium, no panda eyes, no CSF leakage, no focal neurological deficit, <2 episodes vomiting, no amnesia, age <65, no coagulopathy, not struck by motor vehicle, not ejected from motor vehicle, fall <1m.
If seizure choose MRI.
697
What are the indications for MRI?
Demyelination (MS diagnosis)
CNS tumours (no., location, aggressiveness of tumours)
Spine - IV disc prolapse (IV disc degeneration causing neural compression)
TIAs
Epilepsy
Paedatric neurology (e.g. disorders of development, head circumference, congenital malformations)
Headache (e.g. benign intracranial hypertension).
698
What are the contraindications of MRI?
Impaired electronics (most cardiac pacemakers, implantable defies, cochlear implants, relative contraindication for programmable shunts, insulin pumps).
Moveable metabolic implants (aneurysm clips, heart valves, recent intra-abdominal clips).
Relative CIs: pregnancy, claustrophobia, tattoos.
699
What are the indications for angiography?
Aneurysm, arteriovenous malformation, carotidocavernous fistula, unstoppable epistaxis.
700
What are the indications for CT perfusion scan?
Stroke, tumour (increased perfusion indicates angiogenesis and a more aggressive tumour).
701
What is perfusion and how is it measured?
Volume of blood passing through a defined volume of tissue per unit time. Can be CT or MRI. Units: ml blood/100g/min.
702
What are the indications for PET scans?
See increased metabolism seen in tumours, inflammation and infection. Useful after brain tumour resection for differentiating granulation tissue (low energy usage) from leftover tumour (high energy usage).
703
What is PET scanning?
Mapping out of glucose usage over the body.
704
What is an impairment?
Any loss or abnormality of physiological, psychological or anatomical structure or function.
705
What is a disability or activity limitation (AL)?
Any restriction or lack of ability to perfumes an activity in the manner or range considered normal for people of same age, sex or culture.
706
What is handicap/participation restriction?
A disadvantage for a given individual that limits or prevents the fulfilment of a role that would otherwise be normal for that individual.
707
What is rehabilitation?
Restoration of patients to their fullness physical, mental and social capacity.
708
What is rehabilitation medicine?
The speciality of medicine involved with prevention and reduction of activity limitation and participation arising from impairments, and the management of disability from a physical, psychosocial and vocational point of view.
709
What is a long term neurological condition?
Disease or injury to to NS which will affect the individual and their family in one way or another for the rest of their life.
710
What are the different types of LTNCs?
Intermittent/unpredictable, e.g. epilepsy or early MS
Progressive conditions (e.g. NMD, Parkinson's)
Stable conditions, e.g. spina bifida
711
What are some cognitive and psychological issues after brain issue?
Post-traumatic amnesia, confusion, poor concentration, slowed thinking, poor executive functioning, depression, anxiety, personality change, irritability, childishness, selfishness, laziness, behavioural problems, e.g. apathy.
712
What does assessment in rehab involve?
History, exam, mobility, activities of daily living, mood, cognition, bladder, bowels, communication, swallowing, skin, vision, hearing.
713
What does the process of rehab involve?
Problem lists, set goals, formulate management plan, draw upon relevant disciplines, involve patient (and carers).
714
What must goals be?
SMART
| Specific, measurable, achievable, relevant and time limited.
715
What are the benefits of rehab?
Greater independence, greater chance of going or staying home, increased comfort and dignity, greater chance of remaining or returning to work, improved QoL and reduced need for care.
716
What secondary complications can occur when patients are in rehab?
Pressure sores, chest infections, DVT, malnutrition, constipation, MSK pain, contractures, low morale, depression.
717
What specialist services are available at rehab?
Spasticity management, wheelchair and seating services, continence service, sexual/relationship counselling, vocational rehab, orthotics, driving assessment, assessment services for people in low awareness states, pain management, neuropsychiatry/psychology, national behaviour management, ABI service, carers, brain injury group/headway.
718
What is spasticity?
Involuntary motor disorder characterised by velocity dependent increase in tonic stretch reflexes with exaggerated tendon jerks. Disordered sensorimotor control.
719
What causes spasticity?
UMN lesion.
720
What is involved in management of spasticity?
Prevention, MDT, physical therapy, exclude exacerbating factors, oral anti-spasticity agents, focal treatment with botulinum toxin.
721
What are the complications of spasticity?
Poor seating/lying position, sleep difficulties, fatigue, dressing/hygiene issues, pain, spasms and associated reactions, communication and feeding problems, pressure sores and contractures, poor self-image and relationship issues.
722
What are the 5 categories of acquired brain injury?
Head injury (traumatic)
- severe head injury (GCS 3-8, PTA 1-7 days)
- moderate head injury (GCS 9-12, PTA 1-12h)
- mild head injury (GCS 13-15h, PTA <1h).
Haemorrhagic, e.g. SAH
Hypoxic (e.g. out of hospital cardiac arrest)
Metabolic, e.g. hypoglycaemia
Infective, e.g. meningitis
723
What is a radiculopathy?
Radiculopathy leads to a dermatomal sensory loss and motor features corresponding to the affected spinal root. It results from a lesion to the emerging spinal root.
724
What do autonomic signs of cord/root pathology tend to relate to?
Bowel and bladder function.
725
What signs are associated with a C5 cord lesion?
Wasting of C5 innervated muscles, increased tone in lower lumps, reflexes decreased in biceps, all lower reflexes increased, power decreased in C5 innervated muscles and reduced sensation in C5 dermatome.
726
Causes of myelopathy or radiculopathy can be one of two things, name these.
Can be intrinsic - something wrong with the cord or root.
| Can be extrinsic - pressure outside root/cord (tends to be superficial).
727
Give some medical causes of myelopathy?
Inflammation (demyelination (MS), autoimmune, e.g. lupus, sarcoid)
Vascular: ischaemic vs haemorrhagic
Infective (viral (herpes simplex/zooster, EBV, CMV, measles, HIV etc.), bacterial (TB, borrelia, syphilis, brucella), other (schistosomiasis (parasite)).
Metabolic, e.g. B12 deficiency
Malignant/infiltrative
Congenital/genetic (Friedrich's ataxia, spinocerebellar ataxias)
Idioatphic
728
How can occlusion of the central sulcal artery present?
Brown-sequard syndrome.
729
What vertebrae does a spinal stroke tend to affect?
Usually mid thoracic.
730
What is a spinal stroke?
Interruption of blood supply to the spinal cord causing damage to the nerves.
731
Occlusion of what arteries can cause a spinal stroke?
Very rarely posterior spinal artery (in which case, dorsal columns are spared).
Usually anterior spinal artery.
732
What can cause a spinal stroke?
Atheromatous disease (e.g. aortic aneurysm), thromboembolic disease (e.g. endocarditis, AF), arterial dissection (aorta), systemic hypertension, thrombotic haematological disease, hyperviscosity syndromes, vasculitis, venous occlusion, endovascular procedures, decompression sickness, meningovascular syphillis (causes inflammation which blocks BVs).
733
What are the risk factors for spinal stroke?
Vascualr risk factors.
734
Wha are the symptoms of a spinal stroke?
Onset sudden/over several hours.
Back pain/radicular visceral referred pain, usually paraparesis rather than quadraparesis given vulnerability of thoracic cord to flow related ischaemia, numbness, urinary symptoms (retention followed by bladder/bowel incontinence as spinal shock settles).
735
How is a spinal stroke treated?
Reduce risk of recurrence (maintain BP, reverse hypovalaemia/arrhythmias, anti platelet), manage vascular risk factors.
736
Unless significant motor recovery in first ...., chance of major recovery is slow.
24 hours.
737
What are dietary sources of B12?
Meat, fish, animal products, legumes.
738
What complex does the gut require to absorb vit B12?
Intrinsic factor (produced by gastric parietal cells).
739
What most commonly causes B12 deficiency?
Pernicious anaemia.
740
What is pernicious anaemia?
Autoimmune condition in which antibodies bind to intrinsic factor and prevent B12 absorption.
741
How do you investigate B12 deficiency?
FBC/blood film, B12.
742
What else can cause B12 deficiency?
Total gastrectomy, Crohn's, tape worms.
743
What are the signs of B12 deficiency?
Areflexia, first UMN sign - extensor planters, degeneration of corticospinal tracts leads to paraplegia, or of dorsal columns leads to sensory ataxia.
744
What are the symptoms of B12 deficiency?
Paraesthesia in hands and feet, painless retention of urine, loss of pain, proprioception.
745
How is a B12 deficiency treated?
Intramuscular B12 (quicker the better).
746
What are the complications of B12 deficiency?
Myelopathy (L'hermitte's sign), peripheral neuropathy, brain, eyes/optic nerves, brainstem and brainstem problems (can present with blindness, cognitive failure, myelopathy and neuropathy).
747
What is L'hermitte's sign?
Sudden electric shock that passes down spine when flexing neck. Can be due to MS, B12 deficiency and other conditions affecting the spinal cord.
748
What are some common features of neurodegenerative diseases?
Aetiology largely unknown (Mendelian genetic cases rare, often younger onset), usually late onset (disease of elderly), gradual progression, neuronal loss, structural imaging may appear normal or atrophic.
749
What are the types of neurodegenerative diseases?
Dementia
- Alzheimer's
- Vascular
- Lewy Body
- Frontotemporal
- Toxic
- Genetic (Huntington's)
- Infection (HIV, CJD)
- Inflammation (MS)
- Parkinsonism (idiopathic/drug-induced, vascular, plus syndromes.
750
What types of parkinson's plus syndromes are there?
Mulitple system atrophy, progressive supranuclear palsy, corticobasal degen.
751
What are the parkinson's plus syndromes?
A group of neurodegenerative diseases that have the classical features of parkinson's with additional features distinguishing it from idiopathic parkinson's.
752
What is dementia?
A clinical syndrome consisting of progressive impairment of multiple domains of cognitive function in alert patients leading to loss of acquired skills and interference in occupation and social role.
753
How is dementia diagnosed?
Clinical diagnosis (exam and history). Independent witness useful (type of deficit, progression, risk factors, FH.
Exam - cognitive function, neurological and vascular, bloods, CT/MRI, CSF, EEG, functional imaging, genetics biopsy (only if suspect CJD).
IMAGE EVERYONE - don't want to miss rare frontal tumours.
754
How can different clues give indications to what sort of dementia a patient has?
CJD - rapid progression, myoclonus
Vascular - stepwise progression
Abnormal movements - Huntington's, Parkinsonism (Lewy Body).
755
What screening tests can you perform in dementia to gain an idea of neurological status?
Mini-mental (MMSE), montreal (MOCA), neuropsychological.
756
What cognitive domains do you want to assess in dementia?
Memory, attention, language, visuospatial, behaviour, emotion, executive function, apraxias (damage to posterior parietal cortex), agnosias (inability to put meaning on sensory info).
757
What are the symptoms of dementia?
Memory loss, problem with higher cognitive function, may also affect movement and mood.
758
What is dementia managed?
Information/support, dementia services, OT, social work etc.
Melatonin (for insomnia), antipsychotics and anti-depressants.
759
What types of dementia tend to have a younger onset?
Alzheimer's, vascular, frontotemporal, others (toxic (alcohol), Huntington's, HIV, CJD, MS).
760
What types of dementia tend to have a later onset?
Alzheimer's, vascular, lewy body, others.
761
What is alzheimers? What is the mean age of onset? What symptoms are involved?
Most common type of dementia. It is a temporo-parietal dementia. It is progressive.
70yrs.
Early memory disturbance, personality preserved until later.
762
What is the aetiology behind alzheimers disease?
Proteins build up in brain and form plaques. Leads to loss of connections between nerve cells and eventually death of nerve cells and brain tissue. Also leads to loss of chemical messengers in the brain - so signals not transmitted as well.
763
How do you treat alzheimer's +/- Lewy body dementia?
Cholinesterase inhibitors (cholingeric deficit, blocks Each breakdown) - donepezil, rivastigmine, galantine, small symptomatic improvement in cognition (wash out), no delay in institutionalised, NMDA antagonists (memantine) - good for behavioural change, not sedative.
764
What is the aetiology behind vascular dementia?
Caused by reduced blood supply to the brain due to diseased BVs, this leads to lack of brain tissue.
765
How do you treat vascular dementia?
Aspirin to decrease risk of stroke but no evidence for cognitive effect. No good evidence for decreasing vascular risk factors.
766
What is frontotemporal dementia?
Frontal and temporal lobes damaged leading to probelms with behaviour, problem solving, planning and control of emotions.
Leads to early change in personality/behaviour (apathetic, loss of motivation, change in eating habits). Early dysphagia, memory and visuospatial relatively preserved.
767
What is Lewy Body dementia?
Shares symptoms of Alzheimers and parkinson's. Lewy bodies are tiny deposits of proteins that appear in nerve cells of brain.
768
What are the treatable causes of dementia?
B12 deficiency, thyroid disease, HIV, syphillis.
769
What are the mimics of dementia?
Hydrocephalus, tumour, depression (pseudo dementia).
770
What is Parkinson's syndrome?
A clinical syndrome consisting of 2 or more of:
- Bradykinesia
- Rigidity
- Tremor (asymmetric and at rest)
- Postural instability
771
How do you investigate parkinsonism?
Functional imaging (dopamine transporter SPECT - insert radioactive substances that will bind reversibly to DAT transporters. In Parkinson's initially one putamen reduce, then both, followed by reduction of caudate.
772
What is the pathophysiology of dementia?
Pathology in basal ganglia, predominantly dopamine loss.
773
What are the causes of Parkinsonism?
Idiopathic Parkinson's disease (overlap with dementia with Lewy body), drug induced (e.g. dopamine antagonists (anti-psychotics), vascular Parkinsonism (multiple mini strokes that hit the basal ganglia), Parkinson's plus syndromes (ANS hit, early bowel and bladder problems).
774
Is Parkinsonism quickly or slowly progressing?
Slow (5-10 years).
775
Does Parkinsonism respond well to treatment? What is involved in treatment?
Yes - very good response to dopamine replacement (levodopa, COMT inhibitor (entacapone), dopamine agonists (ropinirole, pramipexole, rotigotine), MAO-B inhibitor (seleglline, rasaglline, safinamide).
776
What is involved in late treatment of Parkinsonism?
Drugs to prolong levodopa half life (with MAO-B inhibitors, COMT inhibitor, slow release levadopa), add oral dopamine antagonist, continuous infusion (apomorphine, duodena), functional neurosurgery (deep brain stimulation, allied health professionals +/- care package.
777
What are the later complications of Parkinson's?
Drug induced (motor fluctuations (levodopa wears off), dyskinesias (involuntary movements (levodopa), hallucinations, impulse control.
Non-drug induced - depression, BP, bladder, bowel, speech, swallow and balance problems.
778
What are the basic injury types?
Bruises, abrasions, lacerations, incisons, thermal injuries.
779
What are bruises?
Blunt force to skin leading to damage to little BVs and blood flow out into the s/c tissue.
780
Wha are peri-orbital contusions?
Black eyes. Bruising (blood tracts through looser tissues and accumulates there. Due to direct hit to the eye.
Two black eyes - consider injury in head/base of skull.
781
What are abrasions?
Injury to superficial surface of skin, e.g. friction abrasion.
782
What are lacerations?
Tears/splits caused by blunt force. Skin splits at weakest point.
783
What are incisions?
Sharp wound force - true cut.
784
What are the different types of skull fractures?
Ring fracture
Fissure fracture
Depressed fracture (sign)
Hairline fracture
785
What is a ring fracture?
Fracture forms a ring (high energy injury) - can be horizontal/coronal/sagittal ring formed. May involve foramen magnum/sutures.
Where strong bone meet weak bones (e.g. pterion) breaks more likely.
786
What is a fissure fracture?
Less energy (usually blunt force), not enough for ring fracture.
787
What is a depressed fracture?
Indicates a little piece of bone pushed inward by application of force. Sign - as it moulds exactly the shape of weapon.
788
What is a hairline fracture?
Barely visible, often requires no treatment.
