Neurology Flashcards
(285 cards)
1
Q
Name the excitatory neurotransmitters
A
Acetylcholine, noradrenaline, adrenaline, serotonin, dopamine, glutamate, aspartate
2
Q
Name the inhibitory neurotransmitters
A
GABA, histamine and glycine
3
Q
Name the inhibitory/excitatory neuropeptides
A
Vasopressin, ACTH, opioid peptides, ATP, AMP
4
Q
What area of the brain is affected in aphasia
A
left frontal lobe
5
Q
What area of the brain is affected in hemiparesis
A
internal capsule
6
Q
What cranial nerve is affected in Bells palsy
A
12th
7
Q
What is the effect of a destructive cortical lesion in the frontal lobe (either side)
A
Intellectual impairment, personality change, Urinary incontinence, Mono or hemiparesis
8
Q
What is the effect of a destructive cortical lesion in the left frontal lobe
A
Brocas aphasia
9
Q
What is the effect of a destructive cortical lesion in the left tempero-parietal lobe
A
Acalculia, Alexia, Agraphia, Wernickes aphasia, Right-left disorientation, homonymous field defect
10
Q
What is the effect of a destructive cortical lesion in the right temporal lobe
A
Confusional state, homonymous field defect, cant recognise faces
11
Q
What is the effect of a destructive cortical lesion in the parietal lobe (either side)
A
Contralateral sensory loss, Agraphaesthesia, Homonymous field defect
12
Q
What is the effect of a destructive cortical lesion in the right parietal lobe
A
Dressing apraxia, failure to recognise faces
13
Q
What is the effect of a destructive cortical lesion in the left parietal lobe
A
Limb apraxia
14
Q
What is the effect of a destructive cortical lesion in the occipital/occipitoparietal lobe
A
Visual field defects, visuospatial defects, disturbance of visual recognition
15
Q
What is the effect of an irritative cortical lesion in the frontal lobes
A
Partial seizures, focal motor seizures of the contralateral limb, Conjugate deviation of the head and eyes away from the side of the lesion
16
Q
What is the effect of an irritative cortical lesion on the temporal lobes
A
Formed visual hallucination, complex partial seizures, memory disturbance
17
Q
What is the effect of an irritative cortical lesion in the parietal lobe
A
Partial seizures-focal sensory seizures of the contralateral limb
18
Q
What is the effect of an irritative cortical lesion in the parieto-occipital lobe
A
Crude visual hallucinations
19
Q
What is the effect of an irritative cortical lesion in the occipital lobe
A
Visual disturbance (e.g. flashing)
20
Q
What is Brocas aphasia
A
Damage in the left frontal lobe causes reduced speech fluency but preserved comprehension - cant construct sentences but know what they want to say
21
Q
What is Wernickes aphasia
A
Left temporo-parietal damage leaves fluency of language but words are muddled - patients describe finding speech unintelligible
22
Q
What is nominal aphasia
A
Difficulty in naming objects. An early feature in all types of aphasia
23
Q
What is global aphasia
A
Combo of expressive problems of Brocas and loss of comprehension seen in Wernickes. Loss of language production and understanding
24
Q
What is Dysarthria
A
Disordered articulation - slurred speech
25
Causes of anosmia
head injury, tumour (meningioma), URTI, Parkinsons
26
What causes mononuclear field loss
Complete optic nerve lesion
27
What causes bitemporal hemianopia
chiasmal lesion
28
What causes homonymous hemianopia
optic tract lesion
29
What causes homonymous quadrantanopia
temporal or parietal lesion
30
What causes homonymous hemianopia with macular sparing
occipital cortex or optic radiation
31
What causes homonymous hemianopia (hemiscotoma)
Occipital pole lesion
32
What is papilloedema
Swelling of the optic disc
33
Explain afferent pupillary defect (APD)
Complete optic nerve lesion causes a dilated pupil and APD. Pupil is unreactive to light (absent direct reflex) and the consensual reflex is absent. The consensual reflex is preserved when light is shone into the affected eye
34
Explain relative afferent pupillary defect (RAPD)
Incomplete damage to one optic nerve relative to the other. Direct and indirect reflexes are intact but differ in relative strength. When light is swung the left will dilate slightly when illuminated and constrict slightly when right is illuminated (consensual reflex stronger than direct)
35
What can disrupt blood supply in an ischaemic stroke
thrombus or embolus, atheroscleosis, shock, vasculitis
36
What is a transient ischaemic attack
temporary neurological dysfunction lasting less than 24 hours causing by ischaemia but without infarction. Rapid onset of Sx and often resolve before a pt is seen. May precede a stroke
37
What is a Crescendo TIA
Two or more TIA's in a week and indicates a high risk of stroke
38
Are stroke symptoms symmetrical or asymmetrical
Asymmetrical
39
Common stroke Sx
Limb weakness, Facial weakness, Dysphasia, Visual field defect, Sensory loss, Ataxia and vertigo
40
RF for Stroke
Previous stroke or TIA, AF, Carotid artery stenosis, HTN, Diabetes, Raised cholesterol, FHx, Smoking, Obesity, Vasculitis, Thrombophilia, COCP
41
What is the FAST tool
Face, Arm, Speech, Time
42
What is the emergency room tool for stroke
ROSIER - Recognition of stroke in the emergency room. A score of one or more indicates a possible stroke
43
Management of a TIA
Aspirin 300mg daily
Refer for specialist assessment within 24 hours
Diffusion weighted MRI
44
Initial management of stroke
Exclude hypoglycaemia, Immediate CT head to exclude haemorrhage, Aspirin 300mg daily and admit to stroke centre
45
What is given once haemorrhage is excluded in stroke
Thrombolysis with alteplase
46
Alteplase class and moa
Tissue plasminogen activator that rapidly breaks down clots
47
Within how many hours can alteplase be given
4.5 hours
48
Who is considered for thrombectomy
Pt with confirmed blockage of the proximal anterior circulation or proximal posterior circulation and within 24 hours of onset alongside thrombolysis
49
Explain the assessement for the underlying cause of a stroke
Investigate for carotid artery stenosis and AF with carotid imaging and ECG. GIve anticoag if Dx with AF. Surgical interventions for carotid artery stenosis such as carotid endarterectomy, angioplasty and stenting
50
Secondary prevention for stroke
Clopidogrel 75mg once daily, atorvastatin 20-80mg, BP and diabetes control, address modifiable RF
51
Rehab for stroke pt
MDT approach
Stroke physicians, nurses, SALT, dietician, Physio, OT, Social services, Psych, Orthotics
52
What is an extradural haemorrhage
Bleeding between the skull and the dura
53
What is a subdural haemorrhage
Bleeding between the dura and arachnoid
54
What is an Intracerebral haemorrhage
Bleeding into the brain tissue
55
What is a subarachnoid haemorrhage
Bleeding in the subarachnoid space
56
RF for intracranial haemorrhage
Head injury, HTN, Aneurysm, Ischaemic stroke, tumour, thrombocytopenia, Bleeding disorder, Anticoags
57
Presentation of intracranial bleed
Sudden onset headache, seizures, vomiting, reduced consciousness, focal neuro Sx
58
What GCS score warrant airway support
8/15
59
What usually causes an extradural haemorrhage
rupture of the middle meningeal artery in the temporoparietal region or fracture of the temporal bone
60
Shape of extradural haemorrhage
Bi-convex shape limited by the cranial sutures
61
Typical Hx for an extradural haemorrhage
young pt with traumatic head injury and ongoing headache. Period of improved Sx followed by rapid decline as haematoma compresses intracranial contents
62
Cause of subdural haemorrhage
Rupture of bridging veins in the outermost meningeal layer
63
Subdural haemorrhage shape
crescent shape and not limited by cranial sutures
64
Subdural haemorrhage typical patient
elderly and alcoholic pt who have more atrophy in their brains
65
Intracerebral haemorrhage presentation
similar to a stroke with sudden onset focal neurological symptoms such as limb, facial weakness, dysphasia and vision loss
66
Where can intracerebral haemorrhage occur
anywhere in the brain e.g. lobar, deep, intraventricular, basal ganglia, cerebellar
67
Cause of subarachnoid haemorrhage
ruptured cerebral aneurysm
68
Subarachnoid haemorrhage presentation
Sudden-onset occiptal headache during strenuous activity, thunderclap headache description
69
Immediate investigation for intracranial bleed
CT head
70
Initial management of intracranial bleed
Admission to specialist stroke unit, consider surgery, consider intubation/vent/ICU, correct any clotting abnormality (eg platelets or vit K), correct severe HTN but avoid hypo
71
Surgical options for intracranial bleeds
Craniotomy, Burr holes
72
Mortality of subarachnoid haemorrhage
30%
73
RF for subarachnoid haemorrhage
Aged 45-70, women, black ethnicity
74
General RF for Subarachnoid Haemorrhage
HTN, smoking, excessive alcohol intake
75
What is subarachnoid haemorrhage associated with
FHx, Cocaine use, Sickle cell, connective tissue disorder, Neurofibromatosis, Autosomal dominant PKD
76
Presentation of Subarachnoid Haemorrhage
Neck stiffness, Photophobia, Vomiting, Neuro Sx
77
Ix for Subarachnoid Haemorrhage
CT head, blood will cause hyperattenuation in the subarachnoid space
78
Secondary Ix for subarachnoid haremorrhage
LP after 12 hours as bilirubin takes time to accumulate
Sample will show: Raised red cells, Xanthochromia
79
Management of Subarachnoid Haemorrhage
Specialist, intubate/vent, surgical intervention for aneurysm (endovascular coiling, clipping), give nimodipine to prevent vasospasm
80
Management for the complications of subarachnoid haeomorrhage
Hydrocephalus: LP, External ventricular drain, VP shunt
Treat seizures with antiepileptics
81
What is MS
A chronic and degenerative autoimmune condition involving demyelination in the CNS. Immune system attacks myelin sheath of myelinated neurones
82
Typical MS patient
Young adults under 50, more common in women
83
What cells provide myelin in the CNS and PNS
CNS - Oligodendrocytes
PNS - Schwann cells
84
Explain the characteristic pattern in MS
Lesions vary in location, meaning affected sites and Sx change over time. The lesions are described as 'disseminated in time and space'. Early in the disease remyelination occurs and Sx can resolve but this stops in later stages with remyelination being incomplete
85
Causes of MS
Genetic
EBV
Low Vit D
Smoking
Obesity
86
Onset of Sx in MS
Progress over more than 24 hours. Sx last days to weeks at first presentation and then improve
87
What is the most common presentation in MS
Optic neuritis - demyelination of the optic nerve - presents with unilateral reduced vision
88
Key features of optic neuritis
Central scotoma, Pain with eye movement, Impaired colour vision, Relative afferent pupillary defect
89
What are some other causes of optic neuritis
Sarcoidosis, SLE, Syphilis, Measles and mumps, Neuromyelitis Optica, Lyme
90
Tx for optic neuritis
High dose steroids
91
What lesions cause double vision (diplopia) and nystagmus?
Lesions to CN3, CN4 and CN6
92
Where is the lesion in internuclear opthalmoplegia
Medial longitudinal fasciculus
93
Explain internuclear opthalmoplegia
The nerve fibres of the medial internuclear fasciculus connect the CN nuclei that control eye movements. A lesion here causes impaired adduction on the same side as the lesion and nystagmus in the contralateral abducting eye
94
What does a lesion in CN6 cause?
Conjugate lateral gaze disorder
95
What is conjugate lateral gaze disorder
Both eyes look laterally to the left or right, when looking laterally in the direction of the affected eye then the affected eye will not be able to abduct
96
Examples of focal weakness seen in MS
Incontinence, Horners, Facial nerve palsy, Limb paralysis
97
Examples of focal sensory Sx in MS
Trigeminal Neuralgia, Numbness, Paraesthesia, Lhermittes sign
98
What is Lhermittes sign
Electric shock sensation that travels sown the spine into the limbs when flexing the neck. Indicates disease in the dorsal column
99
What is transverse myelitis
A site of inflammation in the spinal cord
100
Explain sensory ataxia
Due to loss of proprioception. This results in a +ve Rombergs test (lose balance when standing with their eyes closed) and can cause pseudoathetosis (involuntary writhing movements). A lesion in the dorsal columns of the spine can cause sensory ataxia
101
What is Clinically isolated syndrome in MS
First episode of demyelination and neuro Sx. Patients may never have another episode or go on to develop MS. MRI lesions can suggest whether they are likely to progress to MS
102
What is Relapsing-Remitting MS?
Most common pattern. Characterised by episodes of disease and Sx and then recovery. The Sx occur in different areas with each episode
103
Classification of relapsing-remitting MS
Active or Worsening:
Active: new Sx are developing or new lesions on MRI
Not Active: No new Sx or MRI lesions
Worsening: Overall worsening of disability over time
Not Worsening: no worsening of disability over time
104
What is secondary progressive MS?
Where there is relapsing-remitting disease but now there is a progressive worsening of Sx and incomplete remission. Sx become increasingly permanent
105
What is Primary progressive MS?
Involves worsening disease and neuro Sx from the point of diagnosis without relapses or remission
106
Investigations to support the diagnosis of MS
MRI shows lesions and LP can detect oligoclonal bands in the CSFe
107
Tx for MS
MDT led. Disease modifying therapies aim to induce long term remission. Relapses treated with steroids (500mg oral for 5 days or 1g IV daily for 3-5 days)
108
Symptomatic Tx for MS
Exercise, Treat fatigue with amantidine/modafinil/SSRI, Neuropathic pain management, Depression treat with SSRI, Urge incontinence with antimuscs (solifenacin), Spasticity with baclofen or gabapentin, Oscillopsia with gabapentin or memantine
109
What is MND
Encompasses a variety of specific disease that affect the motor nerves. It is progressive and eventually fatal
110
Name the types of MND
Amyotrophic Lateral Sclerosis, Progressive Bulbar Palsy, Progressive Muscular dystrophy and primary lateral sclerosis
111
What muscles are affected in progressive bulbar palsy
Muscles of talking and swallowing (bulbar muscles)
112
Causes of MND
FHx, smoking, heavy metal exposure, Pesticide
113
Typical MND pt
late middle-aged (60 yo) man possibly with an affected relative
114
Typical onset of Sx in MND
Insidious, progressive weakness of the muscles throughout the body affecting the limbs, trunk, face and speech. Typical is limb first. Clumsiness, dropping things and tripping, slurred speech
115
Signs of LMN disease
Muscle wasting, reduced tone, fasciculations, reduced reflexes
116
Signs of UMN disease
Increased tone and spasticity, Brisk reflexes, Upgoing plantar reflexes
117
Diagnosis of MND
Clinical, made by a specialist when there is certainty
118
Management of MND
Riluzole can slow the progression of the disease and extend survival by several months in ALS. NIV to support breathing
119
Holistic management of MND
Breaking bad news, MDT input, Symptom control (baclofen for muscle spas, Antimusc for excessive saliva), Benzos for breathlessness from anxiety, Advanced directives, EOL care
120
What is Parkinsons
Disease of progressive reduction in dopamine in the basal ganglia leading to a disorder in movement
121
Symptom symmetry in Parkinsons
Asymmetrical
122
Typical Parkinsons Pt
Older man, around 70 with gradual onset of Sx
123
Classic triad in Parkinsons
Resting tremor, Rigidity, Bradykinesia
124
Hertz of Parkinsons tremor
4-6 hertz frequency
125
Describe the tremor in parkinsons
Worse on one side, pill rolling, worse when resting and improves with voluntary movement, worse when distracted
126
Describe the rigidity seen in Parkinsons
Resistance to passive movement of the joint, Tension gives way in small increments, this jerking resistance is termed 'cogwheel rigidity'
127
Effects of bradykinesia
Handwriting gets smaller and smaller (micrographia), Small steps when walking (shuffling gait), Rapid frequency of steps to compensate (festinating gait), Hard to initiate movement, Difficulty turning when standing, reduced facial movements and expressions (hypomimia)
128
Other features of Parkinsons
Depression, Sleep disturbance and insomnia, Loss of sense of smell, Postural instability, Cognitive/memory impairment
129
What is multi system atrophy
A Parkinsons plus syndrome where the neurones in various systems degenerate. can lead to autonomic (postural Hypo, constipation, sweating, sexual dysfunction) and cerebellar dysfunction
130
What is Dementia with Lewy Bodies
Parkinsons plus syndrome. Progressive cognitive decline, associated Sx of visual hallucinations, delusions, REM sleep disorder, fluctuating consciousness
131
Name the two other Parkinsons Plus syndromes
Progressive supranuclear palsy, Corticobasal degenerative
132
Diagnosis of Parkinsons
Clinical
133
What are the four treatment options for Parkinsons
Levodopa, COMT inhibitors, Dopamine agonists, Monamine oxidase-B inhibitors
134
Explain Levodopa therapy
It is a synthetic form of dopamine taken orally. It is usually combined with a peripheral decarboxylase inhibitor (e.g. carbidopa or benserazide) which stops it being metabolised in the body before it reaches the brain. It become less effective over time and is often reserved for when other tx arent working
135
Common combination drugs of levadopa
Co-beneldopa (levadopa + benserazide) and Co-careldopa (Levodopa + carbidopa)
136
SE of Levodopa
Dyskinesia (abnormal movements associated with excessive motor activity) e.g. dystonia, chorea and athetosis
137
Tx for dyskinesia associated with Levodopa
Amantadine (glutamate antagonist)
138
What are COMT inhibitors
E.g. entacapone. Inhibit catechol-o-methyltransferase. The COMT enzyme metabolises levodopa so a COMT inhibitor slows the breakdown of levodopa
139
Explain dopamine agonists
Mimic the action of dopamine. Less effective than Levodopa so are used to delay the use of Levodopa and then used in combo
140
Examples of dopamine agonists
Bromocriptine, Pergolide, Cabergoline
141
SE of Dopamine agonists
Pulmonary fibrosis
142
Explain the use of Monamine oxidase-B inhibitors
Blocks the action of MOA-B enzyme (breaks down dopamine, serotonin and adrenaline). They are more specific to dopamine. Used to delay the use of Levodopa and then in combo
143
Examples of MOA-B inhibitors
Selegiline and Rasagiline
144
Summarise benign essential tremor
Affects voluntary muscles commonly in hands, head, jaw and voice. Symmetrical, 6-12 hertz, improved by alcohol, absent in sleep
145
DDx for benign essential tremor
Parkinsons, MS, Huntingtons, Hyperthyroid, Fever, Dopamine antagonist (antipsychotics)
146
Tx for benign essential tremor
Propranolol
Primidone (a barbituate antiepileptic)
147
What is Epilepsy
Characterised by seizures (transient episodes of abnormal electrical activity)
148
Types of seizures
Generalise tonic-clonic, Partial, Myoclonic, Tonic, Atonic, Absent, Febrile, Infantile spasm
149
Explain tonic-clonic seizures
muscle tensing and jerking associated with loss of consciousness, aka grand mal, before the patient may experience an aura, may be tongue biting, incontinence, groaning and irregular breathing, after the seizure there is a prolonged post-ictal period
150
What are partial seizures
Occur in an isolated area of the brain often in the temporal lobes, affect hearing, speech, memory and emotions. Patient remains awake. They are aware in simple partial and not in complex
151
Symptoms associated with partial seizures
Deja vu, Strange smells/taste/sight or sound, unusual emotions, abnormal behaviour
152
What are myoclonic seizures
Sudden, brief muscle contraction like an abrupt jolt or jump. can occur as part of juvenile myoclonic epilepsy in children
153
What are tonic seizures
Sudden onset of increased muscle tone, where the body stiffens. Results in a fall if standing
154
What are atonic seizures
Drop attacks, sudden loss of muscle tone, may be indicative of Lennox-Gastuat syndrome
155
What are absence seizures
Seen in children, pt becomes blank and stares into space, they are unaware of surroundings and dont respond
156
What are infantile spasms
AKA west syndrome. Rare disorder starting at 6mo. presents with clusters of full body seizures. Hypsarrhythmia is a characteristic EEG finding, poor prognosis and developmental delay
157
Tx for Infantile spasms
ACTH and Vigabatrin
158
DDx for epilepsy
Vasovagal syncope, Pseudosizures, Cardiac syncope, Hypoglycaemia, Hemiplegic migraine, TIA
159
IX for Epilepsy
EEG, MRI brain, ECG, Serum electrolytes, Blood glucose, Cultures
160
Tx for tonic clonic for men and women who cant have children and women who are able to have children
Sodium valproate for men and women who cant have children
Lamotrigine or Levetiracetam for women who can have children
161
Tx for partial seizures for men and women who cant have children and women who are able to have children
Lamotrigine or Levetiracetam for both groups
162
Tx for Myoclonic seizures for men and women who cant have children and women who are able to have children
Sodium valproate for men and women who cant have children
Levitiracetam for women who can have children
163
Tx for tonic and atonic seizures for men and women who cant have children and women who are able to have children
Sodium valproate for men and women who cant have seizures
Lamotrigine for women who can have children
164
Tx for Absence seizures
Ehtosuximide
165
Other less commonly used anti-epileptics
Carbamazepine, Phenytoin, Topiramate
166
Summarise Sodium valproate
Works by increasing the activity of GABA
SE: Teratogenic, Liver damage, hair loss, Tremor, reduced fertility
167
What is status epilepticus
A seizure lasting more than five minutes or multiple seizures without regaining consciousness between them
168
Management of status epilepticus
ABCDE (secure airway, give O2, Check blood glucose, Gain IV acces)
Medical Tx: Benzodiazapine, after 2 doses of benzo give IV Levetiracetam, phenyotin or sodium valproate, third line is phenobarbital or anaethesia
169
What is Allodynia
When pain is experienced with sensory inputs that dont usually cause pain e.g. light touch
170
Which two groups of pain fibres transmit pain, explain
C fibres - transmit signals slowly and produce dull and diffuse pain sensations
A-delta fibres - Transmit signals fast and produce sharp and localised pain sensations
171
Explain the analgesic ladder
Step 1: non-opioid such as NSAID and Paracetamol
Step 2: weak opiate e.g. codeine and tramadol
Step 3: Strong opiate e.g. morphine, oxycodone, fentanyl and buprenorphine
172
SE of NSAIDS
Gastritis, Ulcers, exacerbate asthma, HTN, renal impairment, Coronary artery disease
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SE of Opioids
Constipation, Skin itching, Nausea, Altered mental state, resp depression
174
Typical features of neuropathic pain
numbness, tingling, burning and pins and needles
175
Common cause of neuropathic pain
Post-herpetic neuralgia, nerve damage, MS, Diabetic neuralgia, Trigeminal neuralgia, Complex regional pain syndrome
176
Questionnaire for neuropathic pain
DN4
177
First line Tx for Neuropathic pain
Amitriptyline - a TCA
Duloxetine - SNRI
Gabapentin - anticonvulsant
Pregabalin - anticonvulsant
178
First line for trigeminal neuralgia
Carbamazepine
179
What is facial nerve palsy
isolated dysfunction of the facial nerve and presents with unilateral facial weakness
180
What are the five branches of the facial nerve
Temporal, Zygomatic, Buccal, Marginal mandibular and Cervical
181
Facial nerve motor function
Facial expression, Stapedius, Posterior digastric, Stylohyoid, platysma
182
Facial nerve sensory function
Taste for anterior 2/3 of tongue
183
Facial nerve parasympathetic function
Submandibular and sublingual salivary glands, lacrimal glands
184
UMN vs LMN facial nerve palsy urgency
UMN could be a stroke so its urgent and LMN can be managed less urgently
185
Forehead sparing in facial nerve palsy
Forehead is spared in an UMN lesion so patient can move forehead on the affected side. Not spared in LMN
186
Where do unilateral UMN lesions occur
Stroke and tumours
187
Where do bilateral UMN lesions occur
Pseudobulbar palsies
MND
188
Summarise Bells palsy
Common
Idiopathic
Unilateral LMN facial palsy
Use prednisolone 50mg for 10 days
Lubricating eye drops to prevent eye drying out
189
Summarise Ramsay-Hunt syndrome
Caused by VZV
Unilateral LMN facial nerve palsy
Painful tender vesicular rash in the ear canal, pinna and around ear
Tx with aciclovir and prednisolone and eye drops
190
Infectious causes of LMN facial nerve palsy
Otitis media, Otitis externa, HIV, Lyme
191
Systemic causes of LMN facial nerve palsy
Diabetes, Sarcoidosis, Leukaemia, MS, Guillain-Barré
192
Tumour causes of LMN facial palsy
Acoustic neuroma, Parotid tumour, Cholesteatoma
193
Trauma causes of LMN facial nerve palsy
Direct nerve trauma, Surgery, Base of skull fracture
194
How do brain tumours often first present
Sx of raised ICP
195
Causes of Intracranial HTN
Brain tumour, Intracranial haemorrhage, Idiopathic intracranial HTN, Abscesses or infection
196
In patients with headache what are the concerning features that may suggest raised intracranial HTN
Constant headache, Nocturnal, Worse on waking, worse on coughing or straining, Vomiting, Papilloedema
197
Summarise Papilloedema
Swelling of the optic disc secondary to raised ICP
Raised CSF pressure flows into the optic nerve sheath and causing optic disc to bulge forwards
Seen on fundoscopy as: Blurring of the optic disc margin, Elevated optic disc, Loss of venous palpation, Engorged retinal veins, Patons lines
198
Name the three glial cells
Astrocytes, Oligodendrocytes and ependymal cells
199
What are the three main types of glioma
Atrocytoma (glioblastoma is the most common and aggressive)
Oligodendroglioma
Ependymoma
200
Summarise meningioma
Tumours growing from the cells of the meninges. Usually benign however they take up space which can lead to raised ICP
201
What cancers most often spread to the brain
Lung, Breast, RCC, Melanoma
202
What hormone deficiencies or excesses are caused by pituitary tumours
Acromegaly, Hyperprolactinaemia, Cushings and Thyrotoxicosis
203
Tx of Pituitary tumours
Trans-sphenoidal surgery, Radiotherapy, Bromocriptine to block excess prolactin, Somatostatin analogues
204
What cells are acoustic neuromas associated with
Schwann cells
205
What are bilateral acoustic neuromas associated with
Neurofibromatosis type 2
206
Typical clinical picture in acoustic neuroma
Patient is a 40-60 year old with gradual onset of unilateral sensorineural hearing loss, unilateral tinntius, dizziness and imbalance, sensation of fullness in the ear and facial nerve palsy
207
Management options for acoustic neuroma
Conservative, surgery to remove the tumour and radiotherapy to reduce the growth
208
General management for brain tumour
Surgery, chemo, radio, palliative
209
Huntingtons inheritance and mutations
Autosomal dominant. It is a trinucleotide repeat disorder involving mutations on the HTT gene on chromosome 4 which codes for huntingtin (HTT) protein
210
When do symptoms begin in Huntingtons
30-50 yo
211
What is anticipation
A feature of trinucleotide repeat disorders where successive generations have more repeats in the gene resulting in earlier age of onset and increased severity of the disease
212
Sx in Hungtingtons
Insidious progressive worsening of Sx. Begins with cognitive, psychiatric and mood problems followed by the development of movement disorders. Chorea, dystonia, rigidity, eye movement disorders, dysarthria, dysphagia
213
Diagnosis of Huntingtons
Genetic testing
214
Tx for Huntingtons
Breaking bad news, Genetic counselling, MDT, Physio, SALT, Tetrabenazine for chorea, Antidepressants, Advanced directives, EOLC
215
Prognosis for Huntingtons
Life expectancy is 10-20 years after onset of Sx. as the disease progresses the pt becomes more frail and susceptible to illness (e.g. infections, weight loss, falls and pressure ulcers), Death is often due to aspiration pneumonia or suicide
216
What is Myasthenia Gravis
An autoimmune condition affecting the neuromuscular junction
217
General Sx of MG
Muscle weakness that progressively worsens with activity and improves with rest
218
Typical MG pt
Affects women under 40 and men over 60
219
What tumour is strongly associated with MG
Thymoma - 10-20% of pt with MG have a thymoma
220
What antibodies are involved in MG
Acetylcholine receptor (AChR) antibodies, Muscle-specific kinase (MuSK), Low-density lipoprotein receptor-related protein 4 (LRP4)
221
What muscles are mainly affected in MG
Proximal muscles of the limbs and small muscles of the head
222
Sx and difficulties has by pt with MG
Difficulty climbing stairs, standing from a seat, raising hands above head
Diplopia
Ptosis
Facial movement weakness
Difficulty swallowing
Fatigue in jaw
Slurred speech
223
Ways to elicit muscle weakness in MG
Repeated blinking makes ptosis worse, Prolonged upward gaze worsens diplopia, repeated abduction of arm causes weakness
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Ix for MG
Antibody tests looking for AchR, MuSK, LRP4
CT or MRI of the thymus
Edrophonium test
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What is an edrophonium test
Pt given Iv edrophonium chloride. Normally, cholinesterase enzymes in the NMJ break down acetylcholine, edrophonium blocks these enzymes reducing the breakdown of acetylcholine. as a result the levels of Ach in the NMJ rise which relieves muscle weakness which is suggestive of MG
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Tx for MG
Pyridostigmine - cholinesterase inhibitor that prolongs action of Ach and improves Sx
Immunosuppression (pred or azathioprine) suppresses prod of Ab
Thymectomy
Rituximab
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Explain myasthenic crisis
Causes acute worsening of Sx often triggered by another illness, resp muscles weaken and lead to resp failure. Patients may require NIV or mechanical vent
Tx with IV Ig and plasmapheresis
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What is Lambert-Eaton Myasthenic Syndrome
Similar to MG except the symptoms are more insidious and less pronounced. in most cases it is paraneoplastic occurring alongside small-cell lung cancer. Can occur as a primary condition
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Sx of Lambert Eaton and how they differ from MG
Proximal muscle weakness, Autonomic dysfunction, Reduced or absent tendon reflexes. Sx improve after periods of muscle contraction.
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Management of Lambert Eaton
Exclude underlying malignancy
Amifampridine works by blocking voltage-gated potassium chanells in the presynaptic membrane, which prolongs the depolarisation of the cell membrane and assists calcium channels in carrying out their action
Other: Pyridostigmine, Immunosuppress, IV Ig, Plasmapharesis
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What is Charcot-Marie-Tooth Disease
Inherited disease that affects the peripheral motor and sensory neurones. Causes myelin or axon dysfunction. Majority of mutations are autosomal dominant
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Symptom onset of CMT
Usually start to appear before aged 10 but can be delayed until 40 or later
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Classical features of CMT
High foot arches (pes cavus)
Distal muscle wasting causing "inverted champagne bottle legs"
Lower leg weakness, particularly loss of ankle dorsiflexion (with high stepping gait due to foot drop)
Weakness in the hands
Reduced tendon reflexes
Reduced muscle tone
Peripheral sensory loss
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Other causes of Peripheral neuropathy
ABCDE
A - Alcohol
B - B12 def
C - Cancer (e.g. myeloma) and CKD
D - Diabetes and drugs
E - Every vasculitis
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What drugs cause peripheral neuropathy
Isoniazid, Amiodarone, Leflunomide, Cisplatin
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Management of CMT
There is no cure or treatment to prevent progression. Neuro and genetics made a Dx, Physio, OT, Podiatrists, Analgesia, Ortho surg
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What is Guillain-Barré syndrome
An acute paralytic polyneuropathy that affects the PNS
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Patten of illness in GBS
Acute, symmetrical, ascending weakness and can also cause sensory Sx
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GBS causative organisms
Campylobacter jejuni, CMV and EBV
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GBS pathophysiology
molecular mimicry. B cells of the immune system create Ab against the antigens on the triggering pathogen and these Ab match proteins on the peri neurones causing damage to nerve cells, myelin sheath and nerve axon
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Presentation of Sx in GBS
Usually start within four weeks of the triggering infection. Begin in the feet and progress upwards, peak at 2-4 weeks then recovery. Reduced reflexes. Neuropathic pain. May spread to CN and cause facial weakness. Autonomic dysfunction may lead to urinary retention , ileus and heart arrhythmias
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What criteria tool is used to diagnose GBS
Brighton criteria
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What Ix can support a Dx of GBS
Nerve conduction studies (show reduced signal through the nerves) and LP (raised protein)
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Management of GBS
Supportive, VTE prophylaxis, IV Ig, Plasmapheresis
Resp failure: intubate, ventilate, admit to ICU
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Prognosis for GBS
Recovery can take months to years, most eventually fully recover or are left with minor Sx. Mortality is 5%
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What is neurofibromatosis
A genetic condition that causes nerve tumours (neuromas) to develop throughout the nervous system. They are benign but can cause neurological and structural problems. Type 1 is more common than type 2
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Neurofibromatosis type 1 genetics
Gene found on chromosome 17. Codes for a protein called neurofibramin which is a tumour suppressor protein. Autosomal dominant inheritance
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Features of Neurofibromatosis Type 1
CRABBING:
C - Cafe-au-lait spots
R - Relative with NF1
A - Axillary or inguinal freckling
BB - Bony dysplasia such as Bowing of the long vone or sphenoid wing dysplasia
I - Iris hamartomas
N - Neurofibromas
G - Glioma of the optic pathway
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What are neurofibromas
Skin-coloured, raised nodules or papules with a smooth, regular surface, two or more is signficicant
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Management of NF1
No treatment for the underlying disease, genetic tests are useful, monitor and manage Sx and complications
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Complications of NF1
Migraines, Epilepsy, Renal artery stenosis, Learning disability, Behavioural problems, Scoliosis, vision loss, Malignant peripheral nerve sheath tumours, GI stromal tumours, Brain tumours, Spinal cord tumours, Increased cancer risk
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Summarise NF2
On chromosome 22. Codes for merlin. Schwannomas are associated, autosomal dominant, NF2 is associated with acoustic neuromas
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What is tuberous sclerosis
autosomal dominant genetic condition that affects multiple systems
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Characteristic feature of tuberous sclerosis
Hamartomas which are benign growths. Commonly affect the skin, brain, lungs, heart, kidneys and eyes
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Tuberous sclerosis mutations
TSC1 gene on chromosome 9 which codes for hamartin
TSC2 gene on chromosome 16 that codes for tuberin
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Features of tuberous sclerosis
Ash leaf spots (depigmented areas of skin shaped like ash leaf), Shagreen patches (thickened, dimpled, pigmented patches), Angiofibromas (nose and cheek papules), Ungual fibromas (lumps in nail bed), Cafe-au-lait spots, Poliosis (patch of white hair on head, eyebrows
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Neurological features of Tuberous Sclerosis
Epilepsy, Learning disability, Brain tumours
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Other features of Tuberous Sclerosis
Rhabdomyomas, Angiomyolipoma, Lymphangioleiomyomatosis, Subependymal giant cell astrocytoma, Retinal hamartoma
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Management of tuberous sclerosis
No treatment for gene defect, management is supportive and tx complications. In some circumstances mTOR inhibitors (e.g. everolimus or sirolimus) may be used to suppress the growth of brain, lung or kidney tumours
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Red flags for headaches
Fever, photophobia or neck stiffness (Meningitis, enceph)
New neuro Sx (haemorrhage or tumour)
Visual disturbance (GCA, Glaucoma, tumour)
Sudden onset occipital headache (SAH)
Worse on coughing or straining (Raised ICP)
Postural, worse on standing, lying or bent over (raised ICP)
Vomiting (Raised ICP or CO poisoning)
Hx of trauma (Haemorrhage)
Hx of cancer (met)
Pregnancy (pre-eclampsia)
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Tx for chronic or frequent tension headaches
Amitriptyline
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Tx for prolonged case of sinusitis
steroid nasal spray or antibiotics (phenoxymethylpenicillin)
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When do hormonal headaches occur
Two days before and the first three days of the menstrual period. In the perimenopausal period and in early pregnancy
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Tx for hormonal headaches
Triptans and NSAID's
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What is cervical spondylosis
Caused by degenerative changes in the cervical spine. Causes neck pain, made worse by movement and presents with headaches
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What is Trigeminal Neuralgia
Causes intense facial pain and 90% is unilateral pain. Described as electric shock like, shooting, stabbing, burning, can be triggered by touch, talking, eating, shaving and cold
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Tx for Trigeminal neuralgia
Carbamazepine
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What condition is trigeminal neuralgia more common in
MS
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Four main types of migraines
Migraine wiith aura, without aura, silent migraine (aura but no headache), Hemiplegic migraine
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Five stages of migraine
Permonitory/Prodromal stage, Aura, Headache, Resolution and Postdromal/Recovery
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Typical features of migraine
Unilateral, Moderate-Severe intensity, Pounding or throbbing, Photophobia, Phonophobia, Osmophobia, Aura, N/V
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What is an aura
Visual Sx most common, sparks in vision, blurred vision, lines across vision, loss of visual fields, tingling or numbness, dysphagia
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What is a hemiplegic migraine
Unilateral limb weakness, ataxia and impaired consciousness. Familial hemiplegic migraine is an autosomal dominant genetic condition. Can mimic a stroke or TIA
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Triggers of migraine
Stress, bright lights, Strong smells, Certain foods, Dehydration, Menstruation, Disrupted sleep, Trauma
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Acute management of migraine
NSAID's. Paracetamol, Triptans, Antiemetics
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Explain Triptans
Serotonin receptor agonists and cause cranial vasoconstriction, Inhibit pain signals, Inhibit release of inflammatory neuropeptides
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Contraindications of triptans
HTN, Coronary artery disease, previous stroke
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Prophylactic medications for migraines
Propanolol, Amitriptyline, Topiramate
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Other options for migraine management
CBT, Meditation, Acupuncture, Vitamin B2
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Prophylactic triptans for menstrual migraines
Frovatriptan or zolmitriptan
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What is a cluster headache
Unbearable unilateral headache usually centered around the eye. Occur in clusters of attacks and then disappear
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Typical cluster headache pt
30-50 year old male smoker
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Sx of Cluster headaches
Red, swollen watering eye
Pupil constriction
Eyelid drooping
Nasal discharge
Facial sweating
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Management for cluster headaches
Triptans
high flow 100% O2
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Prophylaxis options for cluster headaches
Verapamil, Occipital nerve block, Prednisolone, lithium
