Neuro Diseases Flashcards
(201 cards)
1
Q
What is the epidemiology of MS?
A
Females
Presentation between 20-40
White populations
2
Q
What is the aetiology/ risk factors for MS?
A
Not understood Exposure to EBV in childhood Living far from equator and low vit D White populations Female
3
Q
Briefly explain the pathophysiology of MS
A
Autoimmune mediated demyelination of olgiodendrocytes.
T cells activate B cells to produce autoantibodies against myelin. Myelin does regenerate but new myelin is less efficient and is temp dependent. Repeated demyelination leads to axonal loss and incomplete recovery= Relapsing and remitting symptoms
There is also sclerosis which further blocks conduction
4
Q
What are the types of MS?
A
Relapsing and remitting (80%)
Secondary progressive
Primary progressive (10-15%)
5
Q
What is the most common type of MS?
A
Relapsing and remitting
6
Q
Where does demyelination normally occur in MS?
A
Optic nerves, around ventricles, corpus callosum, brainstem and cerebellar connections, cervical cord
7
Q
How is MS treated?
A
Advise to live a stress free life Give Vitamin D Acute relapse= IV methyprenisolone Frequent relapse= SC interferon 1B or 1A= Antinflammatory cytokines Monoclonal antibodies= IV alemtuzumab IV natalizumab and dimethyl fumarate Symptomatic treatments= Physiotherapy, bac lofen, botox Stem cell treatments
8
Q
What are the clinical features of MS?
A
Usually presents aged 20-40 Symptoms worsen with heat Pain in one eye upon eye movement Reduced central vision Lhermitte's sign= Tingling into limbs on flex of neck Leg weakness Dysphagia Numbness Trigeminal neuralgia Cerebellar symptoms Urinary incontinance
9
Q
How is MS diagnosed?
A
MRI scan= 95% have periventricular lesions, white matter abnormalities
Bloods to rule out others= FBC, U&Es, HIV test, glucose, inflammatory markers
Must have 2+ attacks affecting different parts of of CNS
Lumbar puncture= IgG bands
Electrophysiology
10
Q
What is the aetiology of meningitis?
A
- Normally= N. Meningitdes (Droplets), strep. pneumoniae, haemophilius influenza
- Pregnancy= Listeria monocytogenes
- Neonates= E. coli, group B haemolytic strep
- Viral= Enterovirus, herpes simplex virus
11
Q
What are the risk factors of meningitis?
A
- Intrathecal drugs
- IV drug abuse
- Immunocompromised
- Elderly
- Pregnant
- Crowding
- Diabetes
- Malignancy
12
Q
What is the clinical presentation of bacterial meningitis?
A
- Headache, fever and neck stiffness
- Sudden onset
- Papilloedema
- Malaise, rigors, photophobia, vomitting, irritability
- Positive Kernig’s and Brudzinskis signs
13
Q
What is the clinical presentation of septicaemia?
A
Non blanching petechial (glass test) and purpuric skin rash, seizures
14
Q
What is the clinical presentation of viral meningitis?
A
- Benign self limiting condition (4-10 days)
- Headaches for months following
15
Q
What is the clinical presentation of chronic meningitis?
A
- Long history of vague symptoms of headache, anorexia, vomitting
- Triad (Headache, neck stiffness, fever) is often absent of late
16
Q
What is the lumbar puncture result of bacterial meningitis?
A
- Cells= Polymorphs
- Protein= Raised
- Glucose= Low
17
Q
What is the lumbar puncture result of chronic (TB) meningitis?
A
- Cells= Lymphocytes
- Protein= Raised
- Glucose= Low/ Normal
18
Q
What is the lumbar puncture result of viral meningitis?
A
- Cells= Lymphocytes
- Protein= Normal
- Glucose= Normal
19
Q
How is meningitis diagnosed?
A
- Blood cultures before lumbar
- Lumbar puncture at L4
- Blood tests= FBC, U&E, CRP
- CT of head for tumour
- Throat swabs
- Don’t perform lumbar if septicaemia is suspected
20
Q
How is meningitis treated?
A
- Bacterial= IV Cefotaxime or IV Ceftriaxone
- IV chloramphenicol if CI with penicilins
- If immunocompromised, add IV amoxicillin to cover listeria
- Consider steroids e.g. dexamethasone to reduced cerebral oedema
- Prophylaxis for contacts
- Viral meningitis= aciclovir
21
Q
What is the cause of extradural haemorrhage?
A
- Most commonly due to a traumatic head injury resulting in fracture of the temporal or parietal bone causing laceration of the middle meningeal artery, typically after temple trauma
22
Q
What are the risk factors for extradural haemorrhage?
A
Usually occurs in young adults
23
Q
What are the clinical features of extradural haemorrhage?
A
- Severe headache, nausea and vomitting, confusion and seizures, weakness and brisk reflexes
- Rapid rise in ICP
- Ipsilateral pupil dilates, coma, bilateral limb weakness, deep and irregular breathing
- Coning
- Death due to respiratory arrest
24
Q
How is extradural haemorrhage treated?
A
- ABCDE emergency management= asses and stabilise patient
- Give IV mannitol if increased ICP
- Refer to neurosurgery
- Maintain airway
25
How is extradural haemorrhage diagnosed?
- CT of head (gold standard)= shows hyperdense haematoma that is biconcave/lemon shaped
- Skull x ray= May be normal or show fracture lines crossing the course of the middle meningeal artery
26
What is the commonest primary headache?
Tension headache
27
What are the causes of tension headache?
- Stress
- Sleep deprivation
- Bad posture
- Hunger
- Eye strain
- Anxiety
- Noise
28
What is the clinical presentation of tension headache?
- Usually has 1 of the following= Bilateral, pressing/tight non-pulsatile, mild/moderate intensity, scalp muscle tenderness
- Band-like tight sensation
- Pressure behind eyes
- Can last 30mins-7days
- Episodic (<15days/month) or chronic (>15days/month)
29
How is tension headache diagnosed?
Clinical diagnosis from history
30
How is tension headache treated?
- Lifestyle advice
- Stress relief e.g. massage, accupuncture
- Symptomatic treatment= aspirin, paracetamol, NSAIDS (but don't use too much, to prevent a medication overuse headache)
31
What is the epidemiology of cluster headache?
- Rarer than migraine
- More common in males
- Affects adults= 20-40yrs
- Commoner in smokers
32
What are the risk factors for cluster headache?
- Smoker
- Male
- Autosomal dominant gene has a role
33
What are the clinical features of a cluster headache?
- Rapid onset of excrutiating pain around one eye, temple or forehead
- Ipsilateral cranial autonomic features= Facial flushing, eye may become watery and bloodshot, blocked nose, miosis +/- ptosis
- Rises to crescendo over minutes and lasts 15-160 mins, once or twice a day, usually at same time
- Episodic clusters last 4-12 weeks and are followed by pain free periods of months or even yrs between clusters
34
How are cluster headaches diagnosed?
Clinical diagnosis= At least 5 headache attacks fulfilling the above criteria
35
How are cluster headaches treated?
- Acute attack: analgesics are unhelpful. Give oxygen for 15 mins
- Triptan = serotonin receptor antagonists- reduces vascular inflammation
- Calcium channel blocker is first line prevention
- Avoid alcohol during cluster period
- Corticosteroids may help during cluster
36
What is the epidemiology of trigeminal neuralgia?
- Peak incidence between 50-60yrs
- More common in females
- Prevalence increases with age
- May be due to genetic predisposition
37
What are the risk factors for trigeminal neuralgia?
- Hypertension
| - Triggers= Washing affected area, shaving, eating, talking, dental work
38
Briefly explain the pathophysiology of trigeminal neuralgia
Compression of the trigeminal nerve by a loop of vein or artery resulting in local demyelination, or due to local pathology (tumours, infarction, MS) resulting in erratic pain signalling
39
What are the clinical features of trigeminal neuralgia?
- Almost always unilateral
- At least three attacks of unilateral facial pain
- Occurs in 1 or more distributions of the trigeminal nerve, with no radiation beyond the trigeminal distribution
- Pain has at least 3 of the following- reoccurring, severe intensity, shooting stabbing or electric pain, precipitated by innocuous stimuli
40
How is trigeminal neuralgia treated?
- Typical analgesics and opioids do not work
- Anticonvulsants can suppress attacks
- Other options include phenytoin, gabepentin, lamotrigine
- May spontaneously remit after 6-12 months
- Surgery may be necessary; microvascular decompression, gamma knife surgery
41
How is trigeminal neuralgia diagnosed?
- At least 3 attacks with unilateral facial pain
| - MRI to exclude secondary causes
42
What is the epidemiology of Herpes Zoster infection?
- 90% of 16 yr olds have been exposed (chickenpox)
| - Shingles can affect all ages but is seen as a disease of the elderly
43
What are the risk factors for shingles?
Increasing age
| Immunocompromised
44
Briefly explain the pathophysiology of shingles
- Latent varicella zoster virus is reactivated in the dorsal root ganglia and travels down the affected nerve via the sensory root in dermatomal distribution
- Results in perineural and intrameural inflammation
- Most commonly occurs in thoracic nerve, and then the ophthalmic division of the trigeminal nerve
45
What are the clinical features of shingles?
- Pain and paraesthesia in dermatomal distribution
- Malaise, myalgia, headache and fever
- Can be over a week before eruption occurs
- Rash- consists of papules and vesicles, causes neuritic pain, crust formation and drying occurs.
46
How is shingles diagnosed?
- Clinical diagnosis
| - Eruption of rash is virtually diagnostic
47
How is shingles treated?
- Oral antiviral therapy begun with 72 hrs of rash onset (Oral aciclovir, oral valiciclovir, oral famcliclovir )
- Topical antibiotic treatment
- Analgesia
48
What are some possible complications of shingles?
- Opthalmic branch of trigeminal nerve (will affect site)
| - Post herpetic neuralgia= burning, intractable pain lasting for over four months, responds poorly to analgesics
49
Briefly explain the pathophysiology of encephalitis?
- Disease which mostly affects frontal and temporal lobes resulting in decreased consciousness, confusion and focal signs
50
What is the aetiology of encephalitis?
- Mainly viral= Herpes simplex, EBV, cytomegalovirus, varicella zoster, HIV
- Non viral= Bacterial meningitis, malaria, TB
51
What are the clinical features of encephalitis?
- Whole brain infected= Problems in higher functioning and consciousness
- Triad= fever, headache, altered mental status
- Begins with features of viral infection= triad, myalgia, fatigue, nausea
- Progresses to: personality and behavioral changes, coma, drowsiness, seizures
- Focal neurological deficit=hemiparesis and dysphagia
- Raised ICP and midline shift
52
How is encephalitis diagnosed?
- MRI: shows area of inflammation and swelling, generally in the temporal lobes in HSV encephalitis. May be midline shifting due to raised ICP
- Electroencephalography (EEG): shows periodic sharp and slow wave complexes
- Blood and CSF serology
- Lumbar puncture shows increased lymphocytes, viral detection by CSF PCR
53
How is encephalitis treated?
- If viral immediate treatment with anti-viral e.g. IV aciclovir- even before the investigation results are available
- Early treatment can reduce mortality and long neuro damage
- Anti seizure medication e.g. primadone
54
What is the epidemiology of subdural haemorrhage?
- Most common in patients with small brain (Babies, elderly and alcoholics)
- Majority of SDHs are from trauma, but can be minor/long ago
- Chronic/ apparently spontaneous SDH is common in elderly
55
What are the causes of subdural haemorrhage?
- Trauma causing bleeding from bridging veins between the cortex and venous sinuses, causing a haematoma between dura and arachnoid mata
56
What are the clinical features of subdural haemorrhage?
- Interval between injury and symptoms can be days-months
- Fluctuating levels of consciousness with insidious physical or intellectual slowing
- Personality change
- Unsteadyness
- Signs of raised intercranial pressure= headache, vomitting, nausea, seizure, raised BP
- Focal neurology (hemiparesis, sensory loss)
- Stupor, coma and coning
57
How is a subdural haemorrhage diagnosed?
CT of head to show diffuse spreading, hyperdense, crescent shaped collection of blood over one hemisphere
58
How is subdural haemorrhage treated?
- Stabilise patient
- Refer to neurosurgeons: Irrigation/ evacuation via burr twist drill and burr hole craniotomy
- Address cause of trauma
- IV mannitol to reduce ICP
59
What are the commonest neoplasms to metastasise to the CNS?
- Non-small cell ling
- Small cell lung
- Breast
- Melanoma
- Renal cell
- GI
60
What are the risk factors for primary brain tumours?
- More common in affluent groups
- Ionising radiation
- Vinyl chloride
- Immunosuppression
- Family history= genetics
61
What are the most common primary brain tumours?
- Gliomas= Astrocytomas or oligodendromas
| - Meningiomas
62
What is the most common primary brain tumour?
Astrocytoma
63
What are the clinical features of brain tumours?
- Progressive focal neurological deficit
- Raised ICP= Headaches, drowsiness, vomitting
- Epilepsy= Focal or generalised
- General cancer symptoms= Weight loss, malaise, anaemia
- Papilloedema
64
How are brain tumours diagnosed?
- CT or MRI to determine size and location of lesions
- Blood tests
- Biopsy via skull Burr Hole
- Positron Emission Tomography
65
How are brain tumours treated ?
- Surgery if less than 75
- Radiotherapy
- Medical treatment of symptoms
- Palliative care
66
What are the two types of stroke?
Ischaemic and haemorrhagic
67
What is the epidemiology of ischaemic stroke?
- Accounts for 80% of all strokes
68
What causes ischaemic stroke?
- Small vessel occlusion/ thrombosis in situ
- Cardiac emboli from AF, MI or infective endocarditis
- Large artery stenosis
- Artherothromboembolism
- Hypoperfusion, vasculitis, hyperviscocity
69
Briefly explain the pathophysiology of ischaemic stroke
Arterial disease and atherosclerosis causes thrombosis to occur which blocks a vessel leading to ischaemia
70
What are the clinical features of ischaemic stroke?
- Cerebral hemisphere: signs contralateral to the affected side, hemiplegia, hemisensory loss, upper motor neurone facial weakness and hemianopia
- Brainstem: Complex, depends on location
- Multi infarct: Multiple steps progressing to dementia
71
What are the causes of CNS bleeds?
- Trauma
- Aneurysm rupture
- Anticoagulation
- Thrombolysis
- Carotid artery dissection
- Subarachnoid haemorrhage
72
What is the epidemiology of haemorrhagic stroke?
- Accounts for 17% of strokes
73
What are the clinical features of haemorrhagic stroke?
- Severe headache= like being hit in the head
- Nausea/ vomitting
- Sudden loss of conscious
- Classic stroke symptoms= signs contralateral to affected. Hemiplegia, hemisensory loss, upper motor neurone facial weakness and hemianopia
74
Briefly explain the pathophysiology of haemorrhagic stroke
- Hypertension resulting in micro aneurysm rupture
- Cerebral amyloid angiopathy: Deposition of amyloid B on the walls of small and medium sized arteries in normotensive patients
- Space occupying lesion e.g. tumour
- Young adults: Carotid/vetebral artery dissection
75
What are the risk factors for haemorrhagic stroke?
Hypertension, excess alcohol, smoking and age
76
How is ischaemic stroke treated?
Aspirin, IV alteplase in at least 4.5 hours (thrombolytic; IV tissue plasminogen activator), antiplatelet (aspirin -> lifelong clopidogrel), maintain glucose, NBM
77
How is ischaemic stroke treated?
Stop anticoagulants.
78
What is the epidemiology of cauda equina syndrome?
- Rare, occuring mainly in adults but can occur at any age
| - Occurs in around 2% of herniated discs
79
What is the aetiology of cauda equina syndrome?
- Herniation of lumbar disc= most commonly at L4/5 or L5/S1
- Tumours/ metastasis
- Trauma
- Infection
- Spondylolisthesis
- Post op haematoma
80
What are the clinical features of cauda equina syndrome?
- Sciatica= pain, numbness and a tingling sensation that radiates from lower back and travels down one leg to the foot
- Saddle anaesthesia
- Bladder/ bowel dysfunction
- Erectile dysfunction
- Variable leg weakness that is flaccid and areflexic (LMN signs)
81
Briefly explain the pathophysiology of cauda equina syndrome
- Nerve root compression caudal to the termination of the spinal cord at L1/2
- Usually large central disc herniations at L4/5 or L5/S1 levels
- Generally S1-S5 nerve root compression
82
How is cauda equina syndrome diagnosed?
- MRI to localise lesions
- Knee flexion- test L5-S1
- Ankle plantar flexion- test S1-S2
- Straight leg raising- L5/S1 root problem
- Femoral stretch test
83
How is cauda equina syndrome treated?
- Refer to neurosurgeon ASAP to relieve pressure
- Microdiscectomy- removal of part of the disc
- Epidural steroid injection
- Surgical spine fixation
- Spinal fusion= reduces pain
84
What causes CN4 palsy?
- Trauma to orbit (rare)
85
What causes CN6 palsy?
- MS, Wernike's encephalopathy, pontine stroke
86
What causes CN8 palsy?
- Skull fracture, toxic drug effects, ear infections
87
What causes CN9 palsy?
- Jugular foreman lesion
88
What causes CN3 palsy?
- Raised ICP, Diabetes, hypertension, giant cell arteritis
89
What causes CN5 palsy?
- Trigeminal neuralgia, herpes zoster, nasopharyngeal cancer
90
What causes CN7 palsy?
- Bells palsy, fractures of petrous bone, middle ear infections, inflammation of parotid
91
What are the symptoms of CN4 palsy?
- Head tilt
| - Diplopia on looking down
92
What are the symptoms of CN6 palsy?
- Adducts eye
93
What are the symptoms of CN8 palsy?
- Hearing impairment
| - Vertigo and lack of balance
94
What are the symptoms of CN9 palsy?
- Gag reflex issues, swallowing issues, vocal issues
95
What are the symptoms of CN3 palsy?
- Ptosis
- Fixed, dilated pupils
- Eyes down and out
96
What are the symptoms of CN5 palsy?
- Jaw deviates to side of lesion, loss of corneal reflex
97
What are the symptoms of CN7 palsy?
- Facial droop and weakness
98
What is a TIA?
- Transient ischaemic attack
| - Rapid onset of neurological dysfunction due to temporary focal cerebral ischaemia without infarct (24 hrs)
99
What is the epidemiology of TIAs?
- 15% of first strokes are preceded by TIA
- More common in males than females
- Black ethnicity are at greater risk
100
What are the clinical features of TIAs?
- Sudden loss of function, usually only for minutes, with complete recovery
- 90% are carotid= Amaurosis fugax (Sudden loss of vision in eye), aphasia, hemiparesis, hemisensory loss, hemianopic visual loss
- 10% are vertebrobasilar= Diplopia, vertigo, vomitting, choking, dysarthria, ataxia, hemisensory lsos, hemianopia, tetraparesis, loss of consciousness
101
What are the risk factors for TIA?
- Age
- Hypertension
- Smoking
- Diabetes
- Heart disease
- Excess alcohol
- Hyperlipidaemia
- Raised packed cell vol
- Combined oral contraceptive
- Clotting disorders
102
What is the ABCD2 score?
The risk of having a stroke after a TIA
103
How is the ABCD2 score calculated?
- Age>60= 1
- BP >140/90= 1
- Unilateral weakness= 2 or Speech disturbance with out weakness= 1
- Symptoms for over an hour= 2 or Symptoms for 10-59 mins= 1
- Diabetes= 1
104
How is TIA diagnosed?
```
- Bloods
= FBC for polycythaemia
= ESR raised in vasculitis
= Glucose for hypoglycaemia
= Creatinine, electrolytes, cholesterol
- ECG for AF or MI
- Carotid artery doppler Ultrasound to look for stenosis/ atheroma
- MR/CT angiography if stenosis to determine extent
```
105
What is the epidemiology of subarachnoid haemorrhage?
- Typical age 35-65
106
What are the risk factors for subarachnoid haemorrhage?
- Hypertension
- Family history
- Diseases that predispose; PKD, Ehlers-Danlos, coarctation of aorta
- Smoking, bleeding disorders, post-menopausal decreased oestrogen
107
What is the aetiology of subarachnoid haemorrhage?
- No cause found= 10%
- Rupture of saccular aneurysm e.g. berry aneurysms= 80%
- Atrioventricular malformations= 10%
108
What are the clinical features of subarachnoid haemorrhage?
- Sudden onset severe occipital headache= Thunder clap
- Vomitting, collapse, seizures often follow
- Depressed levels of consciousness
- Neck stiffness, retinal and vitreous bleed
- Kernig's sign and Brudzinski's sign
- Papilloedema
- Focal neurology
- Marked increase in BP as a reflex
- Sentinel headache in 6%
109
What is a migraine?
- Recurrent throbbing headache for 4-72 hrs
110
What are the types of migraine?
- Aura= Characteristically unilateral. Visual disturbance, photosensitivty, nausea,
- Without aura= Characteristically unilateral, no eyesight disturbances
- Variant= Unilateral motor or sensory symptoms resembling a stroke
111
What are the general migraine clinical features?
At least 2 of; unilateral pain, throbbing-type pain, moderate-severe intensity, motion sensitivity
At least 1 of; nausea/ vomitting, photophobia, normal examination
112
Briefly explain the pathophysiology of migraines?
- Changes in brainstem blood flow cases an unstable trigeminal nerve nucleus
- Release of vasoactive neuropeptides causes neurogenic inflammation
113
What things trigger migraine?
CHOCOLATE
- Chocolate
- Hangovers
- Orgasms
- Cheese
- Oral contraceptive
- Lie ins
- Alcohol
- Tumult
- Exercise
114
How are migraines diagnosed?
- Clinical features
- Neuroimaging; rule out mass lesions
- Lumbar puncture if worried for SAH
115
How are migraines treated?
- Reduce triggers
- Passes in sleep
- Painkillers
- Triptans e.g. sumatriptan
- Can use tricyclic antidepressants, anti convulsants or beta blockers
116
What is giant cell arteritis?
Systemic immune mediated vasculitis affecting medium- large arteries of the aorta
117
What are the risk factors for giant cell arteritis?
- Genetic
| - Age- incidence increases with age
118
What is the epidemiology of giant cell arteritis?
- Primarily in those over 50
- Most common in caucasians
- More common in females
119
Briefly explain the pathophysiology of giant cell arteritis
- Granulomatous arteritis of unknown aetiology affecting in particular the extradural arteries and the large cerebral arteries
- Arteries become inflamed, thickened and can obstruct blood flow
120
What are the clinical features of giant cell arteritis?
- Severe headache
- Tenderness of scalp or temple, claudication of the jaw when eating
- Tenderness and swelling of one or more artery
- Sudden painless vision loss
- Malaise, lethargy, fever, associated symptoms of PMR
- Dyspnoea, morning stiffness and unequal or weak pulses
121
How is giant cell arteritis diagnosed?
- Diagnostic criteria; 3 or more of= over 50, new headache, temporal artery tenderness, ESR raised, abnormal artery biopsy
122
How is giant cell arteritis treated?
- High dose corticosteroids rapidly e.g. oral prednisolone to stop vision loss
123
What is a possible complication of giant cell arteritis?
Polymyalgia rheumatica in 50%
124
What is carpal tunnel syndrome?
The most common mononeuropathy. Results from pressure and compression on the median nerve as it passes through the carpal tunnel
125
What is the epidemiology of carpal tunnel syndrome?
More common in females as women have narrower wrists but similar sized tendons
126
What is the aetiology of carpal tunnel syndrome?
- Usually idiopathic
| - Usually in over 30s
127
What are the clinical features of carpal tunnel syndrome?
- Symptoms are intermittent
- Aching pain in hand and arm
- Paraesthesiae in median nerve distribution
- Relieved by dangling hand over side of bed = Wake and shake
- Wasting of thenar eminence
- Light touch, 2 pt discrimination and sweating
128
How is carpal tunnel syndrome diagnosed?
- Electromyography; See slowing of conduction velocity in the median sensory nerves across the carpal tunnel
- Phalens test= patient can only maximally flex wrist for 1 min
- Tinels test= Tapping on nerve at wrist induces tingling
129
How is carpal tunnel syndrome treated?
- Wrist splint at night
- Local steroid injection
- Decompression surgery
130
What is a cerebrovascular accident?
Syndrome of rapid onset neurological deficit caused by focal, cerebral, spinal or retinal infarct. Lasts more than 24 hrs
131
What is the epidemiology of cerebrovascular accident?
- 3rd most common cause of death in high income countries
- Leading cause of adult disability worldwide
- Rates are higher in Asian and black African populations
- Uncommon in those under 40
- More common in men
132
What are the risk factors for cerebrovascular accident?
- Male
- Black or Asian
- Hypertension
- Past TIA
- Smoking
- Diabetes
- Increasing age
- Heart disease
- Alcohol
- Polycythaemia, thrombophilia
- AF
- Hypercholesterolaemia
- Combined pill
- Vasculitis
- Infective endocarditis
133
How are cerebrovascular accidents diagnosed?
- Urgent CT of head= Infarction is seen as a low density lesion, subtle changes evident within 3 hrs
- ECG, BP monitoring= Look for AF
- FBC to look for thrombocytopenia and polycythaemia
- Blood glucose to rule out hypoglycaemia
134
How are cerebrovascular accidents treated?
- Maximise reversible ischaemic tissue; ensure hydration and o2 stats
- Thrombolysis= Tissue plasminogen activator e.g. IV alteplase, then antiplatelet therapy e.g. clopidogrel 24 hrs later
135
What are the clinical features of peripheral neuropathies?
- Can be asymptomatic
- Diabetes; long history of paresthesia, pain, weakness, wasting, incontinence, sexual dysfunction
- Foot ulcer
136
What are the 6 mechanisms that cause peripheral neuropathies?
1. Demyelination
2. Axonal degeneration
3. Compression
4. Infarction
5. Infiltration
6. Wallerian degeneration
137
What is the aetiology of peripheral neuropathies?
- Guillain Barre syndrome
- Diabetes
- Alcohol
138
How are peripheral neuropathies diagnosed?
- Nerve conduction studies
- FBC
- Diabetes screen
139
How are peripheral neuropathies treated?
Diabetic control, Amitriptyline for neuropathic pain
140
What is Guillain-Barre syndrome?
An acute inflammatory demyelinating ascending polyneuropathy affecting the peripheral nervous system following an upper resp tract or GI infection
141
What are the clinical feature of Guillain-Barre syndrome?
- 1-3 weeks post infection, a symmetrical ascending muscle weakness starts
- Lost reflexes
- Some paraesthesia
- Little to no muscle wasting
142
What are the risk factors for Guillain-Barre syndrome?
- History of resp or GI infections, 1-3 weeks prior to onset
- Vaccinations have been implicated
- Post pregnancy
143
What causes Guillain-Barre syndrome?
- Antibodies to an infection in GI or resp tract attacks the peripheral nerves
- Usually caused by C. jejuni or CMV
144
How is Guillain-Barre syndrome diagnosed?
- Clinical
- Antibody screen
- Nerve conduction studies
- CSF at L4: Raised proteins with normal WCC
- Monitor FVC
145
How is Guillain-Barre syndrome treated?
- IV immunoglobulins for 4 days
- Plasma exchange possible
- Ventilate if needed
- Low molecular weight heparin e.g. SC enoxaparin, and compression stockings to reduce thrombotic risk
146
What is myasthenia gravis?
Autoimmune disease against nicotinic acetyl choline receptors in neuromuscular junction
147
What are the clinical features of myasthenia gravis?
- Weakness, fatigability of ocular, bulbar and proximal limbs
- Improves after rest
- Look for ptosis, diplopia and myasthenic snarl on smiling
- Resp difficulties
148
Briefly explain the pathophysiology of myasthenia gravis
Autoantibodies to nicotinic acetylcholine receptors or MuSK at the post-synaptic membrane of the neuromuscular junction, causing receptor blockade/loss
149
What are the causes of myasthenia gravis?
- Under 50= Associated with other autoimmune diseases
| - Over 50= Thymic hyperplasia or thymic tumour
150
What is the epidemiology of myasthenia gravis?
- Women more than men
- Peak age of incidence at 30 in women
- Peak age of incidence at 60 in men
151
How is myasthenia gravis diagnosed?
- Anti-AChR antibodies in serum (incerased in 90%)
- Electromyography or nerve stimulation tests
- Ice test; improvement of ptosis with ice
- CT of thymus to look for hyperplasia, tumour etc
152
How is myasthenia gravis treated?
- Anticholinesterases; pyridostigmine
- Immunosuppressants if anticholinesterases don't work; prednisolone or azathioprine
- Thymectomy
153
What is a myasthenia crisis?
Weakness of the respiratory muscles. Treat with plasmapheresis and IV immunoglobulins
154
What is Huntington's disease?
Autosomal dominant neurogenerative, loss of GABA and Ach, but dopamine spared.
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What is Chorea?
A continuous flow of jerky, semi purposeful movements. May interfere with voluntary movements but cease during sleep
156
What are the clinical features of Huntington's disease?
- Relentlessly progressive
- Chorea
- Personality changes
- Dysarthria, dysphagia and abnormal eye movements
- Associated with seizures
- Later, dementia
- Behavioural symptoms
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Briefly explain the pathophysiology of Huntington's disease?
Presence of mutant huntingtin protein leads to loss of neurones in the caudate nucleus and putamen of the basal ganglia. This leads to depletion of GABA and ACh
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What is the epidemiology of Huntington's disease?
- Usually onset in middle age
- Autosomal dominant condition with full penetrance
- Very rare
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What is the aetiology of Huntington's disease?
- Autosomal dominant
| - CAG repeats in Huntingtin protein gene on chromosome 4
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How is Huntington's disease diagnosed?
- Genetic testing
| - CT/MRI= Shows caudate nucleus atrophy and increased size of the frontal horns of lateral ventricles
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How is Huntington's disease treated?
- Treat chorea symptoms= Benzodiazepine, anticonvulsants, muscle relaxant, sodium valproate
- Genetic counselling
- Antidepressants such as SSRIs
- Antipsychotic agents
- Risperidone for aggression
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What are the risk factors for Huntington's disease?
Family history
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What is epilepsy?
The recurrent tendancy to spontaneous, intermittent, abnormal electrical activity in part of the brain, manifesting in seizures/
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What are the types of epilepsy?
- Generalised tonic clonic (Grand mals)
- Absence (Petite mals)
- Myoclonic, tonic and akinetic
- Partial
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What are the risk factors for epilepsy?
- Family history
- Premature born babies
- Abnormal vessels in brain
- Alzheimer's or dementia
- Use of drugs e.g. cocaine
- Stroke/ brain tumour/ infection
166
Briefly explain the pathophysiology of epilepsy
Uncontrolled electrical activity in the brain. Innervation of muscle fibres can cause physical movements and sensory disturbance
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What is generalised tonic clonic epilepsy?
- Sudden onset of rigid tonic phase, followed by convulsion (clonic) phase/
- Tongue biting, incontinence of urine, drowsiness/ coma
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What is absense epilepsy?
- Usually in childhood
| - Caese activity, stares and pales
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What is myoclonic, tonic and akinetic epilepsy?
- Muscle jerking (myoclonic), intense stiffening (Tonic) or cessation of movement
- Falling and loss of consciousness (Akinetic)
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How is epilepsy diagnosed?
- To make a clinical diagnosis, there needs to be at least 2 unprovoked seizures >24 hrs apart
- Short term video EEG
- CT, MRI
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How is generalised tonic clonic epilepsy treated?
- AED: Sodium valproate
- Lamotrigine
- Carbamazepine
- Diazepam for seizure control
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How is absence epilepsy treated?
- AED: Sodium valproate
- Lamotrigine
- Ethosuximide
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What is the first line treatment for partial epilepsy?
- AED: Lamotrigine or carbamazepine
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What are the types of motor neurone disease?
- Amyotrophic lateral sclerosis (most common)= UMN + LMN
- Primary lateral sclerosis= UMN
- Progressive muscular atrophy= LMN
- Progressive bulbar palsy= UMN+ LMN of the lower cranial nerves
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What are the clinical features of motor neurone disease?
- Depends on type
- Upper limbs; Reduced dexterity, stiffness, wasting of intrinsic muscles of the hands
- Lower limbs; Tripping, stumbling gait, foot drop
- Bulbar; Slurred speech, hoarsness, dysphagia
- Muscular atrophy and spasticity
- Never affects eye movements or sensory
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What causes motor neurone disease?
- Unknown
| - Family history= Mutations in free radical scavenging enzyme copper/zinc superoxide dismutase
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What is the epidemiology motor neurone disease?
- Relatively uncommon
- Middle age
- More common in men
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How is motor neurone disease diagnosed?
- Clinical= 3 or more LMN+UMN signs
- EMG: Muscle degeneration
- Brain/ cord MRI= Helps to exclude structural causes
- Lumbar puncture to exclude inflammatory causes
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How is motor neurone disease treated?
- Sodium channel blocker; Riluzole
- Symptomatic treatment=
Spasms; Baclofen
Drooling; Propantheline
Analgesia; NSAIDs such as diclofenac
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What is Parkinson's disease?
Degeneration movement disorder caused by reduction in dopamine in the substantia nigra
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What is the epidemiology of Parkinson's disease?
- Increasing prevelance with age
- Peak age of onset at 35-65 yrs
- More common in males
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What causes Parkinson's disease?
- Genetic links
| - Some environmental link; less common in smokers
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What are the clinical features of Parkinson's disease?
- Gradual onset of symptoms= asymmetrical
- Starts with impaired dexterity or unilateral foot drop
- Anosmia, depression, aches, REM sleep disorders, urinary urgency, hypotension
- Rigidity, bradykinesia, and resting tremor
- Pill rolling tremor
- Speech becomes quiet, indestinct and flat
- Drooling
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Briefly describe the pathophysiology of Parkinson's disease
Progressive loss of dopamine secreting cells from the substantia nigra causes an alteration in neural circuits in the basal ganglia. Thought to be due to abnormal accumulation of alpha-synuclein bound to ubequitin= lewy bodys in the cytoplasm
185
How is Parkinson's disease diagnosed?
- Clinical
- Can confirm with response to levodopa
- MRI = intially normal, but will show atrophy of substantia nigra
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How is Parkinson's disease treated?
- Gold standard= Levodopa alongside a decarboxylase inhibitor e.g. co-careldopa
- Dopamine agonist
- MAO-B inhibitors; selegiline
- Deep brain stimulation
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What is the most common cause of acute compression of the spinal cord?
Vertebral body neoplasms (commonly from lung, breast, kidney, prostate or thyroid)
188
How are spinal cord compressions diagnosed?
- MRI (Gold standard)
| - Biopsy/ surgical exploration may be required
189
What is the epidemiology of Alzheimer's Disease?
Onset may be as early as 40 yrs (and even earlier if patient has Down's syndrome)
190
What are the causes of Alzheimer's Disease?
- Environmental and genetic
- Accumulation of beta-amyloid peptide
- Defective clearance of beta-amyloid plaques
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What are the risk factors for Alzheimer's Disease?
- 1st degree relative
- Down's syndrome
- Homozygous for apolipiprotein e
- Hypertension, diabetes, hyperlipidaemia, AF
- Decreased activity
- Depression and loneliness
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What are the clinical features of Alzheimer's Disease?
- General features of dementia
- Insidious onset with steady progression
- Short term memory loss, disintergreation of personality
- Decline in language, visuospatial skills and agnosia
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How is Alzheimer's Disease treated?
- Acetylcholinesterase inhibitors e.g. rivastagmine
- Antiglutamatergic treatment= Memantine
- Folic acid and B vitamins
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What are the types of dementia?
- Alzheimers (50%)
- Vascular (25%)
- Lewy-body (15%)
- Fronto-temporal
- Parkinsons
- Mixed
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What are the risk factors for dementia?
Family history, age, Down's syndrome, alcohol use, obesity, high blood pressure, diabetes, hypercholesterolaemia, atherosclerosis, depression, high oestrogen
196
What are the clinical features of vascular dementia?
Stepwise deterioration with declines followed by short periods of stability. History of TIA or strokes.
197
Briefly explain the pathophysiology of vascular dementia
Brain damage due to cerebrovascular disease; either a major stroke, multiple smaller unrecognised strokes or chronic changes in smaller vessels
198
Briefly explain the pathophysiology of Lewy-body dementia
Deposition of abnormal protein within neurons in the brain stem and neocortex
199
What are the clinical features of Lewy-body dementia?
- Fluctuating cognition with pronounced variation in attention and alertness
- Memory loss in later stages
- Impairment in attention, visuospatial ability is prominent
- Depression and sleep disorders
- Parkinson's
200
How is dementia diagnosed?
- History; assess cognitive functions
- Mini mental state exam (24 or less is impairment in cognition)
- Exclude rarer causes of dementia (B12 deficiency, thyroid fucnction tests, HIV)
- MRI to see extent of atrophy
201
How is dementia prevented?
- Healthy behaviours
- Smoking cessation
- Good diet
- Physical activity
- Mental activities
- Leisure activities
- Education
