Neurology Notes Flashcards
(798 cards)
1
Q
How long do TIAs last
A
Less than 24 hours
2
Q
What is the ABCD2 score used for?
A
Assessing a patient who has had a TIAs subsequent risk of having a stroke (Age, Blood Pressure, Clinical Features (unilateral weakness, dysphasia without weakness, Duration, Diabetes)
3
Q
Treatment for stroke where intracranial haemorrhage has been excluded
A
Thrombolysis with IV Alteplase, as long as within 4.5 hours
4
Q
What is the gold standard imaging technique for stroke
A
Diffusion weighted MRI
5
Q
What are 4 examples of secondary prevention of Stroke?
A
- clopidogrel 75mg daily
- atorvastatin
- carotid endarectomy or stenting
- treat modifiable RF
6
Q
At what point on the GCS should airways be secured
A
8/15
7
Q
What is the pathophysiology of subdural haemorrhage
A
Rupture of bridging veins
8
Q
How does a subdural haemorrhage appear on a CT scan
A
Crescent shape and are not limited by the cranial sutures
9
Q
What is the pathophysiology of an extra Dural haemorrhage
A
Rupture if the middle meninges artery in the temporal-parietal region (can be associated with fracture of the temporal bone)
10
Q
What is the shape of an extra dural haemorrhage on a CT scan
A
Bi Convex shape and limited by the cranial sutures
11
Q
What are risk factors (other than aneurysm) for SAH
A
Cocaine, sickle cell anaemia
12
Q
What are the features of essential tremour?
A
- postural: worse if arms outstretched
- improved by alcohol and rest
- strong family history
13
Q
What should be investigated in essential tremour?
A
Check U + Es, calcium, LFT, TFT (checks for other causes)
14
Q
What is the inheritance of essential tremour?
A
Autosomal dominant
15
Q
What is the management of essential tremor?
A
Propranolol (first line), primidone
16
Q
What are the triad of symptoms of Parkinsons?
A
Rest tremor, rigidity, bradykinesia
17
Q
What conditions can present similarly to PArkinsons?
A
Supranuclear palsy, normal pressure hydrocephalus, multiple system atrophy, Wilson’s disease, post-encephalitis
18
Q
How can you differentiate supranuclear palsy to parkinsons?
A
Will present additionally with gaze issues, swallowing issues, early falls
19
Q
What drugs can cause Parkinsonisms?
A
Anti-psychotics, metoclopramide
20
Q
Why does domperidone not cause extra-pyramidal side effects?
A
Doesn’t cross the blood brain barrier
21
Q
Why do Parkinsons patient get chorea?
A
Due to medication (over stimulation of basal ganglia). Typically levodopa
22
Q
Why does Wilson’s disease cause Parkinsonism?
A
Copper is mostly deposited in the basal ganglia (particularly putamen and globus pallidus)
23
Q
Outside of the typical triad, what presentation can Parkinsons have?
A
- depression (common)
- sleep disturbance
- anmosia
- mask like facies
- micrographia
- drooling of saliva
- sleep disturbance
- fatigue
- autonomic dysfunction, such as postural hypotension
24
Q
What type of sleep disturbance occurs in Parkinsons?
A
REM sleep behaviour disorder. Can occur many years before diagnosis
25
What sort of tremor is seen in Parkinsons?
Pill rolling
26
When does the tremor in Parkinsons improve and worse?
It is more noticeable when resting and improves on voluntary movement. It gets worse when the patient is distracted. Performing a task with the other hand (e.g., miming the act of painting a fence) exaggerates the tremor
27
What frequency is the tremor in Parkinsons?
4-6 hertz
28
What sort of rigidity is seen in Parkinsons?
Cogwheel
29
What is hypomimia? What condition is it seen in?
Reduced facial movements and facial expressions. Seen in Parkinsons
30
What are the main side effects of levodopa?
Dyskinesia (abnormal movements associated with excessive motor activity)
31
What advantage does dopamine agonists have over levodopa?
Less dyskinesia
32
What Parkinsons medication offers the most improvement in motor symptoms?
Levodopa
33
What is the distribution of Parkinsons symptoms?
Asymmetrical
34
What is the pathophysiology of Parkinsons?
Progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra. Reduction in dopamine output results in the classical triad of symptoms.
35
What autonomic disturbances are seen in Parkinsons?
Postural hypotension, urinary frequency, hypersalivation
36
What surgical treatment can be given for Parkinsons?
Deep brain stimulation: electrodes stimulate basal ganglia
37
Are symptoms sudden or gradual onset in Parkinsons?
Gradual
38
What ways can bradykinesia present in Parkinsons?
- handwriting getting smaller (micrographia)
- small steps when walking (shuffling gait)
- rapid frequency of steps to compensate for small steps and avoid falling (festinating gait)
- difficulty initiating movement
- reduced facial movements + expressions (hypomimia)
39
What worsens/improves essential tremors?
Alcohol improves, rest improves. Worsens with intentional movement, worsens when arms outstretched
40
What is multiple system atrophy?
condition where neurones of various systems in the brain degenerate, including basal ganglia
41
What is the presentation of multiple system atrophy?
Parkinson's presentation, autonomic dysfunction, cerebellar dysfunction (-> ataxia)
42
What are Parkinson's plus syndrome?
Multiple system atrophy, dementia with Lewy bodies, progressive supranuclear palsy, corticobasal degeneration
43
What is a dementia associated with Parkinsons?
Lewy body dementia
44
How can lewy body dementia and Parkinsons be differentiated?
Cognitive symptoms start before motor symptoms in Lewy body dementia. Other way round in Parkinsons
45
How can Lewy body dementia be differentiated to other forms of dementia?
- fluctuating cognition
- early impairments in attention and executive function rather than just memory loss
- visual hallucinations
- Parkinsonism
46
What are the two types of multiple system atrophy?
Predominant Parkinsonian features and predominant cerebellar features
47
What condition does Parkinsonism with associated autonomic symptoms indicate?
Multiple system atrophy
48
What autonomic disturbance is often seen as an early feature of multiple system atrophy?
Erectile dysfunction
49
How is Parkinsons typically diagnosed?
Clinically: history and examination findings. By a specialist in movement disorders
50
What is the first line treatment in Parkinsons patients, where motor symptoms are affecting quality of life?
Levodopa
51
What is the first line treatment in Parkinsons patients, where motor symptoms are NOT affecting quality of life
Dopamine agonist, MAO-B inhibitor
52
Of the antiparkinsonism drugs, which offers the most improvements in motor symptoms and ADLs?
Levodopa
53
Which Parkinsons drug has the most motor complications?
Levodopa
54
Which Parkinsons drug has the most associated adverse events?
Dopamine agonists (excessive sleepiness, hallucinations, impulse control disorders)
55
What are common s/e of levodopa?
Dry mouth, anorexia, palpitations, postural hypotension, psychosis
56
What drug is levodopa nearly always given with?
Decarboxylase inhibitor (carbidopa)
57
Why is levodopa usually given with a carboxylase inhibitor?
This prevents the peripheral metabolism of levodopa to dopamine outside the brain, which can reduce side effects
58
What are examples of dopamine receptor agonists?
Bromocriptine, ropinirole, cabergoline, apomorphine
59
What are ergot derived dopamine receptor agonists?
Bromocriptine, cabergoline
60
What s/e are ergot derived dopamine agonists associated with, and what monitoring should occur?
Associated with pulmonary, retroperitoneal, cardiac fibrosis. Thus, prior to starting do a ECHO, ESR, creatinine and CXR. Requires careful monitoring
61
What are possible s/e of dopamine agonists?
Impulse control disorders, hallucinations, excessive daytime sleeping
62
How does MAO-B inhibitors work for Parkinsons treatment?
Inhibits the breakdown of dopamine secreted
63
How can dosage of Levodopa change over time?
May have to increase, as it becomes less effective over time
64
What are examples of dyskinesia due to Levodopa?
- dystonia (excessive muscle contraction leads to abnormal postures/exaggerated movements)
- chorea
- athetosis (involuntary twisting/writhing movements)
65
When is amantadine used in Parkinsons?
Used to reduce the dyskinesia associated with levodopa
66
What drug class is amantadine?
Glutamate antagonist
67
What is the action of COMT enzyme in Parkinsons/Levodopa tx?
Metabolises levodopa in the body and brain
68
Why are COMT inhibitors given?
To extend the effective duration of levodopa; inhibits the breakdown of the drug
69
What drug is entacapone?
COMT inhibitor
70
What are examples of MAO-B inhibitors?
Selegiline, rasagiline
71
What Parkinsons drug has the highest risk of impulse control disorder?
Dopamine agonist therapy
72
What medication can be used to treat excessive salivation in Parkinsons?
Glycopyrronium bromide
73
What is the role of anti muscarinics in Parkinsonisms?
Treat drug induced parkinsonism, can help tremor and rigidity. Procyclidine also in acute dystonia in anti psychosis medication
74
What muscles are affected by benign essential tremor?
Voluntary muscles: head, jaw, vocal tremor
75
What are possible causes of tremors?
Parkinsonism, essential tremor, anxiety, thyrotoxicosis, hepatic encephlopathy, CO2 retention (CO2), cerebellar disease, drug withdrawal
76
What age does MND rarely present before?
40 years
77
What are the types of MND?
ALS, primary lateral sclerosis, progressive muscular atrophy, progressive bulbar palsy
78
What is the most common type of MND?
ALS
79
How does progressive bulbar palsy typically present?
Palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei
80
What type of MND has the poorest prognosis?
Progressive bulbar palsy
81
What type of MND has the best prognosis?
Progressive muscular atrophy
82
What is the typical distribution of signs in ALS?
LMN signs in arms, UMN signs in legs
83
What is the most common presentation of ALS?
Asymmetric limb weakness
84
What disease should fasciculations make you consider?
MND
85
What is the presentation of MND?
- asymmetric limb weakness
- mixture of LMN and UMN
- wasting of small hand muscles/tibialis anterior
- fasciculations
- absence of sensory signs/symptoms
86
What muscles are characteristically spared in MND?
External ocular
87
What investigations can support a dx of MND?
Nerve conduction studies, MRI, electromyography
88
Why is nerve conduction studies done in MND? What is the typical result?
Exclude a neuropathy. Show normal motor conduction
89
What is the typical results for electromyography in MND?
Reduced number of action potentials with increased amplitude
90
Why is MRI done in MND?
To exclude the differential diagnosis of cervical cord compression and myelopathy
91
What are the potential differential dx in MND?
Neuropathy, cervical cord compression, myelopathy
92
What are the cognitive domains?
Memory, visuospatial, language, executive function, social cognition/behavioural, attention
93
What are symptoms of episodic memory loss?
reptititive in conversation, limited knowledge of current affairs, may forget appointments
94
What are signs of episodic memory loss?
Cannot recall breakfast, cannot recall pictures from naming test, Ribot's phenomenoma
95
What is Ribot's phenomenoma
More recent memories lost more than remote ones
96
What are symptoms of visuospatial memory loss?
Difficulty reading unusual fonts, optician visits, misperceptions, hallucinations, loss of confidence driving
97
What are signs of visuospatial memory loss?
Unable to draw clock faces or intersecting pentagons, prosopagnosia (can't recognise faces)
98
What is symptoms of language memory loss?
Forgetting words, stumbling over words, neologisms, vague descriptions, asking what things are/mean
99
What are signs of language memory loss?
Unable to repeat difficult words, phrases or follow complex commands. Unable to read unusual words, unable to demonstrate use of object
100
What is the symptoms of executive function loss?
Disorganised, unable to multitask, frequently distracted, lacking initiative, odd decisions made
101
What are signs of executive function loss?
Poor estimates, impaired letter fluency, stroop test
102
What are symptoms of social cognition loss?
Loss of empathy, inappropriate behaviour, callous, self obsessed, humourless, preference for sweet foods (lol)
103
What is praxis?
Conception, planning, executive of a specific action (eg, gait apraxia)
104
What are causes of rapid onset dementia?
CDJ, vasculitis, limbic encephalitis, space occupying lesion, seizures, infections (HIV, HSV), metabolic
105
What are LMN signs?
Muscle wasting, reduced tone, fasciculations, reduced reflexes
106
What are UMN signs?
Increased tone or spasticity, brisk reflexes, upgoing plantar reflexes
107
What is used in the treatment of MND?
Riluzole, non invasive ventilation (BiPAP)
108
What is the MoA of riluzole?
Prevents stimulation of glutamate receptors
109
What conditions has association with epilepsy?
Cerebral palsy, tuberous sclerosis, mitochondrial diseases
110
What age does febrile convulsions typically occur between?
6 months and 5 years
111
What are the features of a typical febrile convulsion?
Early in viral infection as temperature rises rapidly, brief, tonic-clonic
112
What are the features of a simple febrile convlusion?
<15 minutes, generalised seizure, no recurrence in 24 hours, complete recovery within an hour
113
What are the features of a complex febrile seizure?
15-30 minutes, focal, repeated seizures
114
What is the management following a paediatric febrile seizure?
Children who have had a first seizure OR any features of a complex seizure should be admitted to paediatrics
115
When should a parent ring an ambulance in a febrile seizure?
If it has lasted longer than 5 minutes
116
Do antipyretics reduce the chances of a febrile seizure?
No
117
What is the treatment for recurrent febrile seizures?
BDZ rescue medication (Rectal diazepam or buccal midazolam)
118
What are focal siezures?
Start in a specific area, typically on one side of the brain
119
Do patients remain conscious in generalised seizures?
No, all patients become immediately unconscious
120
What are the types of generalised seizure?
Tonic-clonic, tonic, clonic, typical absence, myoclonic, atonic
121
What is West's syndrome?
Infantile spasms in the first few months of life
122
How do infantile spasms present?
Flexion of the head, trunk, and limbs. This is followed by extension of the arms
123
What typically causes West's syndrome?
Neurological abnormality
124
What is the typical features of juvenile myoclonic epilepsy?
Infrequent generalised seizures, often in the morning. Daytime absences, and sudden shock like myoclonic seizures. Worse after drinking alcohol
125
What is the treatment of juvenile myoclonic epilepsy?
Sodium valproate
126
How can post ictal phases differentiate seizures and syncope?
Following a seizure: patients have a post ictal phase where they feel drowsy and tired for around 15 minutes. Syncopal episodes have quicker recovery
127
What symptoms may be associated with epilepsy?
Tongue biting, incontinence of urine
128
What type of seizures of seen in febrile convulsion?
Tonic/tonic-clonic
129
What features may be seen in temporal seizures?
Epigastric aura and automatism, deja vu, jamais vu, less commonly hallucinations
130
What features may be seen in frontal lobe partial seizures?
Head/leg movements, posturing, post ictal weakness, Jacksonian march
131
What is the difference between tonic, clonic, myoclonic and atonic?
Tonic: muscle tensing
Clonic: muscle jerking
Myoclonic: brief rapid muscle jerks
Atonic: less of muscle tone
132
Do eyes shut or stay open in an epileptic seizure?
Typically remain open
133
What are the differential diagnoses for epileptic seizures?
Vasovagal syncope, pseudoseizures, cardiac syncope, hypoglycaemia, TIA
134
When are AEDs started?
After second epileptic seizure
135
How can AEDs affect the P450 system?
Many epileptics can induce/inhibit the P450 system resulting in varied metabolism of other medications, for example warfarin
136
What are the possible s/e of sodium valproate?
P450 enzyme inhibitor, increased appetite and weight gain, alopecia, tremor, hepatitis, pancreatitis, thrombocytopaenia
137
What is the MoA of sodium valproate?
Increases GABA activity
138
When is sodium valproate used?
Generalised seizures in men (teratogenic)
139
What is the MoA of carbamazepine?
Binds to sodium channels, increasing refractory period
140
What are the possible s/e of carbamazepine?
P450 enzyme inhibitor, dizziness and ataxia, drowsiness, agranulocytosis, visual disturbance
141
What is the possible s/e of lamotrigine?
Stevens Johnson syndrome
142
What is the first line treatment of seizure? What time is it administered?
5-10 minutes, give benzodiazepine
143
When is status epilepticus?
>5 mins
144
What is the first line treatment for generalised seizures (men vs women)?
Men: sodium valproate
Women: lamotrigine or levetiracetam
145
What is the treatment for partial seizures (men vs women)?
Both lamotrigine or levetiracetam
146
What is the treatment of absence seizures?
Ethosuximide
147
Are eyes open or closed in a seizure?
Open. Closed in syncope
148
What benzodiazepine is given in status epilepticus?
Pre-hospital: PR diazepine or buccal midazolam
Hospital: IV lorazepam
149
How many doses of IV lorazepam can be given in status epilepticus?
Maximum two doses
150
What is second line for status epilepticus?
Levetiracetam, phenytoin, sodium valproate
151
Is consciousness affected in focal seizures?
No, not impaired
152
Can mothers on AEDs breastfeed?
Yes
153
What birth defects can sodium valproate be associated with?
Neural tube defects
154
What is the prognosis of benign rolandic epilepsy?
Excellent; most will outgrow by adolescence?
155
What is the cause of Lateral medullary syndrome?
Occlusion of the posterior inferior cerebellar artery
156
What are the symptoms of lateral medullary syndrome?
Ipsilateral facial pain and temperature loss, contralteral limb/torso pain, ataxia (cerebellar signs), nystagmus
157
What are the typical symptoms of neuromalignant syndrome?
Pyrexia, muscle rigidity, autonomic lability (HTN, tachycardia, tachypnoea), agitated delirium with confusion, raised creatine kinase, AKI, leukocytosis
158
What is the pattern of damage for the radial nerve?
Wrist drop, sensory loss to area between dorsal aspect of the 1st and 2nd metacarpals
158
What type of fracture puts the radial nerve at risk?
Shaft fracture of the humerus
159
What is the treatment of Bell's palsy?
Prednisolone
160
What is MS?
A chronic cell mediated autoimmune disorder characterised by demyelination in the CNS
161
What gender is MS more common in?
Women
162
What is the most common form of MS?
Relapsing remitting disease
163
What is a very common (non specific) presentation of MS?
Lethargy
164
What are common visual presentations of MS?
Optic neuritis, optic atrophy, Uhthoff's phenomenon
165
How does optic neuritis present?
Pain, unilateral vision loss, visual field loss, loss of colour vision, flashing lights, central scotoma (enlarged central blind spot)
166
What is Uhthoff's phemomenom?
Worsening of vision and other symptoms following rise in body temperature
167
What are the sensory features of MS?
Pins/needles, numbness, trigeminal neuralgia, Lhermitte's syndrome
168
What is Lhermitte's syndrome?
Parasethesiae in limbs on neck flexion
169
What motor symptom is seen in MS?
Spastic weakness (most commonly in legs), limb paralysis
170
What general symptoms are seen in MS? (not motor, sensory or visual)
Urinary incontinence, sexual dysfunction, intellectual deterioration
171
What is required for a diagnosis of MS?
Demonstration of lesions disseminated in time and space
172
What is the pathophysiology of MS?
Myeline covers axons of neurones, helps the travel of electrical impulses. In MS, inflammation damages myelin. In early stages, re-myelination can occur and symptoms can resolve. Further on in disease, this happens less.
173
Does MS affect central or peripheral nervous system?
Central (the oligodendrocytes)
174
What are the differential diagnosis of optic neuritis?
Sarcoidosis, SLE, syphilis, measles, mumps, Lyme disease, MS
175
What is the treatment of optic neuritis?
High dose steroids
176
Lesions in what cranial nerves cause double vision and nystagmus?
Oculomotor (III), trochlear (IV), abducens (VI)
177
What is internuclear ophthalmoplegia?
Nerve fibres of the medial longitudinal fasciculus connect the cranial nerve nuclei that control eye movements and ensure the eyes move together.
178
Where is the lesion that causes internuclear ophthalmoplegia?
Medial longitudinal fasciculus
179
What is the presentation of internuclear ophthalmoplegia?
Causes impaired adduction on the same side as the lesion and nystagmus in the contralteral eye
180
What causes conjugate lateral gaze disorder?
Lesion in the abducens nerve
181
How does conjugate lateral gaze disorder present?
When looking laterally in the direction of the affected eye, the affected eye will not be able to abduct. For example, in a lesion involving the left eye, when looking to the left, the right eye will adduct (move towards the nose), and the left eye will remain in the middle.
182
What is the difference in sensory and cerebellar ataxia?
Sensory: due to loss of proprioception
Cerebellar: due to problems from the cerebellum coordinating movement
183
What test comes back positive in sensory ataxia?
Rombergs (lose balance with eye's closed)
184
Where is the lesion in ataxia?
Dorsal columns of the spine
185
How is MS diagnosed?
MRI (can demonstrate lesions) or lumbar puncture (detect oligoclonal bands in the CSF)
186
How is acute relapse treated in MS?
High dose steroids (oral or IV methylprednisolone)
187
What are drug options for reducing risk of relapse in MS?
Natalizumab, ocrelizumab, fingolimod, beta interferon
188
What is first line drug treatment for reducing the risk of relapse in MS?
Natalizumab (recombinant monoclonal antibody)
189
What is the first line treatment of spasticity in MS?
Baclofen and gabapentin
190
What additional investigation should patients with bladder dysfunction in MS undergo and why?
Ultrasound to assess bladder emptying: if there is significant residual volume, anticholinergics may worse
191
What is the treatment for bladder dysfunction in MS?
Anti cholinergics
192
What is the treatment of fatigue in MS?
Amantadine (1st line: unknown mechanism), modafinil or SSRI
193
What are the features of an ataxic gait?
Wide based, falls, cannot walk heel to toe
194
What are the cerebellar causes of an ataxic gait?
MS, posterior fossa tumour, alcohol, phenytoin toxicity
195
If there is right ataxia and the cause is cerebellar, where will the cerebellar lesion be?
Same side
196
What are the proprioceptive causes of an ataxic gait?
Sensory neuropathies (low B12), inner ear problem (affecting vestibular system)
197
What are the mechanisms that blood supply to the brain can be disrupted?
Thrombus/embolus, atherosclerosis, shock, vasculitis
198
What is the onset time of a stroke?
Rapid
199
What is the main risk factor for an embolic stroke?
AF
200
What are the risk factors for haemorrhagic stroke?
Age, HTN, AVM, anticoagulation therapy
201
What are the typical features of a stroke?
Asymmetrical. Limb weakness, facial weakness, dysphasia, visual field defects (homonymous hemianopia), sensory loss, ataxia and vertigo
202
What specific symptoms are seen in brainstem infarcts?
Severe stroke symptoms including quadroplegia and lock-in-syndrome
203
What are the specific symptoms of cerebral hemisphere infarcts?
Contralateral hemiplegia (initially flaccid then spastic), contralateral sensory loss, homonymous hemianopia, dysphasia
204
What are the symptoms of lacunar infarcts?
Pure sensory stroke OR ataxic hemiparesis OR unilateral weakness (and/or sensory deficit)
205
What are lacunar infarcts?
Small infarcts around the basal ganglia, internal capsule, thalamus, pons
206
What is the Bamford classification?
Classifies strokes based on the initial symptoms
207
What are the criteria for the Bamford classification?
1. Unilateral hemiparesis and/or hemisensory loss of the face, arm and leg
2. Homonymous hemianopia
3. Higher cognitive dysfunction (eg, dysphasia)
208
What arteries are involves in total anterior circulation infarcts?
Middle and anterior cerebral arteries
209
What criteria of the Bamford classification is present in a total anterior circulation infarct?
All 3
210
What arteries are affected in partial anterior circulation infarcts?
Smaller arteries of anterior circulation
211
What arteries are affected in lacunar infarcts?
Arteries around the internal capsule, thalamus and basal ganglia
212
What type of stroke is likely in a patient who presents with isolated unilateral hemiplegia without speech or vision problems?
Lacunar infarct
213
What arteries are affected in posterior circulation infarcts?
Vertebrobasilar arteries
214
What symptoms are more likely in a haemorrhagic stroke?
Decrease in level of conciousness, headache, nausea and vomiting, seizures
215
When can patients be offered thrombolysis?
Confirmed ischaemic stroke, and within 4.5 hours of stroke symptoms. IV alteplase given
216
What is the management of TIA?
Immediate 300mg aspirin unless C/I
217
What is the associated affects with anterior cerebral artery stroke?
Contralateral hemiparesis (weakness) and sensory loss, more often lower extremity
218
What is the most and least common location of stroke infarcy?
MCA= most common
ACA = least likely
219
What is the symptoms of MCA infarct?
Contralateral hemiparesis and sensory loss, more often upper body. Hemiplegia. Aphasia (Broca's)
220
What blood supply has infarcted if there is Broca's aphasia?
MCA
221
Where is Broca's located?
Frontal lobe
222
How is LMN and UMN distinguished in a patient with facial palsy?
Forehead sparing will have UMN origin (due to bilateral innervation)
223
What commonly causes LMN facial paralysis?
Bell's palsy
224
What is the presentation of a stroke in PCA region?
Visual issues: often contralateral homonymous hemianopia with macular sparing
225
What is Weber's syndrome?
Midbrain stroke syndrome
226
What is the presentation of Weber's infarct?
Ipsilateral CN III palsy (down and out), contralateral weakness of upper and lower extremity
227
What is the presentation of anterior inferior cerebellar artery occlusion?
Ataxia, nystagmus. Ipsilateral: facial paralysis and deafness. Contralateral limb/torso pain and temperature loss
228
What is seen in ophthalmic artery infarct?
Amaurosis fugax
229
What is seen in basilar artery infarct?
Locked in syndrome
230
Which area of the brain affected by a stroke which causes expressive dysphasia?
Broca's area (dominant hemisphere, usually left)
231
Which area of the brain would a stroke cause receptive dysphasia?
Wernicke's
232
What should be prescibed alongside steroids?
PPI (steroids can cause gastric irritation)
233
How would you assess someone's risk of a stroke if they have AF?
CHADSVASC
234
What are the general clinical features of a posterior circulation infarct?
Vertigo, imbalance, unilateral limb weakness, slurred speech, double vision, headache, N+V, cerebellar signs
235
What are the features of a total anterior circulation infarct?
Cognitive impairment, speech troubles, unilateral weakness/incoordination, unilateral numbness or loss of sensation, dysarthria, unilateral visual loss, loss in 1 visual field
236
What arteries are involves in posterior circulation infarcts?
vertebrobasilar arteries
237
What should be given as soon as a haemorrhagic stroke has been excluded?
300mg aspirin
238
What is the target time that thrombectomy should be offered within?
6 hours
239
What imagine should be done prior to thrombectomy and why?
Computed tomographic angiography (CTA) or magnetic resonance angiography (MRA). This is to confirm which vessel is infarcted
240
What vessel area is always offered thrombectomy if within timeframe?
proximal anterior circulation
241
When would thrombectomy be done if more than 6 hours have passed?
If there is potential to salvage brain tissue as shown by CT perfusion or diffusion weighted MRI
242
What is first line secondary prevention of a stroke?
Clopidogrel
243
What is second line secondary prevention of a stroke?
Aspirin plus MR dipyridamole
244
When is carotid artery endartectomy recommended?
If they have had a stroke/TIA in the carotid territory, not severley disabled, and have >70% carotid stenosis
245
What investigations are done after a stroke to identify if a specific risk factor caused the event?
- carotid imaging: identify carotid artery stenosis
- ECG: identify AF
246
What is a validated tool that assesses stroke in hospital?
ROSIER score
247
What should be immediately excluded if a patient is presenting with a possible stroke?
Hypoglycaemia
248
What is assessed as part of the ROSIER tool?
Loss of consciousness or syncope, seizure activity (these make stroke LESS likely). Points if new, acute: asymmetric facial weakness, asymmetric arm weakness, asymmetric leg weakness, speech disturbance, visual field defect
249
What is the first line investigation for suspected stroke?
non contrast CT head scan
250
What will be seen on a non contrast CT head scan if there is an acute ischaemic stroke?
Hyperdense artery
251
What will be seen on a non contrast CT head scan if there is a haemorrhagic stroke?
Area of hyperdensity surrounded by low density (oedema)
252
What is the first line agents used to control BP in a patient post stroke? Why?
intravenous labetalol, nicardipine and clevidipine as first-line agents, due to the possibility for rapid and safe titration to control blood pressure
253
What should be done to blood pressure prior to thrombolytic therapy in stroke patients?
Reduced to 185/110, as elevated BP can affect thrombolytic eligibility
254
What is the treatment of TIA?
Immediately give 300mg aspirin
255
What is the management of a patient with a suspected TIA who has a bleeding disorder or taking an anticoagulant?
Admit immediately to exclude haemorrhage
256
When do TIA symptoms typically resolve?
Within an hour
257
When should a patient who has had a suspected TIA in the last 7 days be reviewed by a specialist?
Within 24 hours
258
When should a patient who has had a suspected TIA which has occurred more than a week ago by reviewed by a specialist?
Within 7 days
259
When should a patient who has had more than 1 TIA or has a suspected cardioembolic source or severe carotid stenosis by reviewed?
Discuss need for admission/observation urgently.
260
What neuroimaging is recommended in TIA patients?
MRI (including diffusion weight and blood sensitive sequences)
261
Is CT done for a TIA?
Generally no, unless there is clinical suspicion of an alternative diagnosis
262
What imaging should all TIA patients have done urgently, and why?
Carotid doppler, due to risk of carotid artery atherosclerosis
263
What is the first line treatment for TIA patients?
Clopidogrel, after initial aspirin therapy
264
What secondary therapy should be given to TIA patients, aside for antiplatelet?
High intensity statin
265
What layers of the brain do subarachnoid haemorrhages occur between?
The pia mater and arachnoid membrane
266
What is associated with SAH risk?
Cocaine use, FHx, sickle cell anaemia, connective tissue disorders, neurofibromatosis, autosomal dominant polycystic kidney disease
267
What is the first line investigation for a SAH?
Non contrast CT head
268
Is traumatic or spontaneous SAH more common?
Traumatic
269
What are the causes of spontaneous SAH?
Intracranial aneurysm (berry), AVM, pituitary apoplexy
270
What are conditions associated with berry aneurysms
HTN, adult polycystic kidney disease, ehlers danlos, coartication of the aorta
271
What are the features of SAH?
Thunderclap headache (severe, occipital), potential sentinel headache, nausea and vomiting, meningism, coma, seizures, ECG changes
272
What ECG changes might be seen during SAH?
ST elevation
273
Should a LP be done if CT head is negative within 6 hours of suspected SAH?
No: consider alternative dx
274
What course of action should be taken if CT head is done >6 hours after SAH and is normal?
Do a lumbar puncture
275
What are LP results consistent with a SAH?
Xanthichromia (RBC break down), normal or raised opening pressure
276
What time should LP be done within suspected SAH?
<12 hours
277
If a SAH is confirmed via CT, what should be the next steps of investigation?
Identify causative pathology via CT intracranial angiogram (to identify vascular lesion)
278
What medication is used to prevent vasospasm after a SAH?
Nimodipine
279
What is the risk of confirmed aneurysmal SAH?
Risk of rebleeding: most treated by interventional coil clipping, and some craniotomy and neurosurgical clipping
280
What is the investigation of potential aneurysmal SAH rebleeding?
Repeat CT
281
What are the potential complications of SAH?
Rebleeding, hydrocephalus, vasospasm, hyponatraemia, seizures
282
How is hydrocephalus after SAH treated?
External ventricular drain or long term VP shunt
283
What electrolyte imbalance is commonly seen after SAH and why?
Hyponatraemia due to SIADH
284
What most commonly causes a extradural haematoma?
Low impact trauma
285
What is the typical presentation of a patient with an extradural haematoma?
Patient who initially loses, briefly regains, and then loses consciousness after a low impact head injury
286
What pupillary sign is seen in patients with ED haematoma and why?
Fixed and dilated pupil due to the compression of the parasympathetic fibres of the third cranial nerve as the brain swells
287
What is the definitive treatment of ED haematoma?
Craniotomy and evacuation of the haematoma
288
What are the three types of subdural haemorrhage?
Acute, subacute, chronic
289
What is an acute subdural haematoma?
Collection of fresh blood within the subdural space
290
What are subacute subdural haematomas associated with?
High impact injuries, therefore often associated with other underlying brain injuries
291
What are the surgical options for an acute subdural haematoma?
Monitoring of intracranial pressure and decompressive craniectomy
292
What causes chronic subdural haematoma?
Rupture of the small bridging veins, causes slow bleeding
293
What is the time frame for symptoms developing in chronic subdural haematoma?
Typically several weeks or a month after
294
How do acute vs chronic subdurals appear on CT?
Acute: hyperdense (bright)
Chronic: hypodense (dark)
295
What are the indications for operating on a chronic subdural?
If the patient is confused, has an associated neurological deficit or severe imaging findings
296
What is the surgery of choice in chronic subdural haematoma?
Surgical decompression with burr holes
297
What are the features of intracranial venous thrombosis?
Slow onset headache, nausea and vomiting, reduced consciousness, diplopia, facial pain, signs of raised intracranial pressure
298
What is the investigation of choice for intracranial venous thrombosis?
MRI venography. Additionally d-dimer may be raised
299
What is the management of intracranial venous thrombosis?
Anticoagulation with LMWH acutely, warfarin in longer term
300
What sign is seen on venography with sagittal sinus thrombosis?
Empty delta sign
301
What are the risk factors for intracranial venous thrombosis?
Smoking, COC pill, hx of clots
302
What are factors that favour pseudoseizures?
Pelvic thrusting, family member with epilepsy, female, crying after seizure, don't occur when alone, gradual onset
303
What blood test may be elevated after a true seizure, which can help differentiate from pseudoseizures?
Serum prolactin
304
What is the inheritance of Duchennes?
X-linked recessive
305
How does Duchennes typically present?
With a child who has vague symptoms of muscle weakness, and uses their hands on their legs to help them stand up (Gower's sign)
306
What blood test may come back raised in Duchenne's?
Creatinine kinase
307
What protein is the mutation in with Duchennes + Beckers?
Dystrophin
308
What is myopathy?
A disease that affects muscles that control voluntary movement in the body; patients experience muscle weakness
309
What are the typical features of myopathies?
Symmetrical muscle weakness (proximal > distal), common problems are rising from a chair/getting up stairs/rushing hair.
Sensation + reflexes normal
310
What are the potential causes of myopathy?
Polymyositis, Duchenne/Becker dystrophy, myotonic dystrophy, Cushing's, thyrotoxicosis, alcohol
311
What is Bell's palsy?
Facial nerve paralysis
312
Is Bell's palsy uni or bilateral?
Unilateral
313
Is Bell's palsy UMN or LMN?
LMN
314
What is the features of Bell's palsy?
Entire facial paralysis (including the forehead). Can also have altered taste, dry eyes, hyperacusis (reduced tolerance to sound)
315
What is the treatment of Bell's palsy?
Oral prednisolone within 72 hours, and eye care (artificial tears and eye lubricants)
316
When should patient's with Bell's palsy be referred?
No improvement after 3 weeks, refer to ENT
317
What are the 5 branches of the facial nerve?
Temporal, zygomatic, buccal, marginal mandibular, cervical
318
What is the facial nerve pathway?
Exits the brainstem at the cerebellopontine angle. On its journey to the face it passes through the temporal bone and parotid
319
What is the motor function of the facial nerve?
Facial expression, stapedius in the inner ear
320
What is the sensory function of the facial nerve?
Taste to anterior 2/3 of the tongue
321
What is the parasympathetic function of the facial nerve?
Supplies the submandibular, sublingual, and lacrimal glands
322
What can cause bilateral UMN lesions?
Pseudobulbar palsies, MND
323
What causes Bell's palsy?
It is generally idiopathic, though there may be links with some viral causes
324
What is Ramsay-Hunt syndrome?
Reactivation of the varicella zoster virus in the geniculate ganglion of the 7th cranial nerve
325
What is the presentation of Ramsay Hunt syndrome?
Unilateral LMN facial nerve palsy, with the addition of a painful + tender vesicular rash in and around the ear. Can also have vertigo and tinnitus
326
What is normally the first feature of Ramsay Hunt syndrome?
Auricular pain
327
What is the management of Ramsay Hunt syndrome?
Oral aciclovir and corticosteroids, lubricating eye drops
328
What structures does the facial nerve supply?
Structures of the second embyronic branchial arch
329
What are causes of bilateral facial nerve palsy?
Sarcoidosis, guillain barre syndrome, lyme disease, bilateral acoustic neuromas (as in neurofibromatosis typ 2), Bell's palsy (though normally unilateral)
330
What are the possible causes of unilateral facial nerve palsy?
Sarcoidosis, GBS, Lyme disease, acoustic neuroma, Bell's palsy, Ramsay-Hunt syndrome, parotid tumours, MS, HIV, diabetes mellitus, trauma (direct nerve trauma, base of skull fractures)
331
What are the features of carbon monoxide poisoning?
Headache, N+V, vertigo, confusion, weakness
332
What is the key investigation in patients with potential carbon monoxide poisoning?
Carboxyhaemoglobin levels
333
When might carboxyhaemoglobin be falsely raised?
In smokers (<10%, vs <3% in non smokers)
334
What are the three branches of the trigeminal neuralgia?
Ophthalmic, maxillary, mandibular
335
What types seizures will carbamazepine worsen?
Absence or myoclonic seizures
336
What are the possible causes of trigeminal neuralgia?
Idiopathic mostly, but also potentially due to compression of the trigeminal roots by tumours or vascular problems
337
What are the triggers for pain in trigeminal neuralgia?
Light touch: such as washing, shaving, smoking, talking, brushing teeth
338
What is the pain experienced in trigeminal neuralgia?
Unilateral. Brief, electric shock like pains, abrupt in onset and termination, limited to one or more divisions of the trigeminal nerve
339
What are trigger areas in trigeminal neuralgia?
Small areas of the nasolabial fold or chin
340
What are red flag symptoms in trigeminal neuralgia that may suggest a serious underlying problem?
Sensory changes, deafness, pain only in the opthalmic division of the nerve (eye socket, forehead, nose), bilateral pain, optic neuritic, FHx of MS, onset before 40 years
341
What is the first line treatment for trigeminal neuralgia?
Carbamazepine
342
What condition frequently is co-concurrent with trigeminal neuralgia?
MS
343
What is myasthenia gravis?
An autoimmune condition affecting the neuromuscular junction
344
What age are patients typically with myasthenia gravis?
Men: over 60
Women: under 40
345
What condition does myasthenia gravis have a strong link with?
Thymomas
346
What is the pathophysiology of myasthenia gravis?
Acetylcholine receptor (AChR) antibodies bind to the postsynaptic acetylcholine receptors, blocking them and preventing stimulation. Therefore, the more receptors are used during muscle activity, the more they become blocked. The antibodies also activate the complement system at the neuromuscular junction, which leads to cell damage
347
Which gender is more commonly affected by myasthenia gravis?
Women
348
What antibodies are seen in myasthenia gravis?
ACh-AB, MuSK (muscle specific kinase antibodies), low density lipoprotein receptor related protein 4 antibodies (LRP4)
349
What is the function of MuSK and LRP4 normally, and why does this lead to M.G?
MuSK and LRP4 are important proteins for the creation and organisation of the acetylcholine receptor. Destruction of these proteins leads to inadequate acetylcholine receptors
350
What is the typical presentation of myasthenia gravis?
Muscle fatigability (worsen on activity, improve on rest), extraocular muscle weakness (diplopia), proximal muscle weakness, ptosis, dysphagia, jaw fatigue, slurred speech
351
What set of disorders is M.G associated with?
Autoimmune conditions (pernicious anaemia, autoimmune thyroid disorders, SLE, rheumatois)
352
What are methods for eliciting fatigability in muscles in M.G examination?
Repeated blinking exacerbates ptosis, prolonged upward gazing exacerbates diplopia
353
What are the investigations for myasthenia gravis?
Single fibre electromyography (nerve conduction studies: action potentials decrease after repeated stimulation), CT thorax to exclude thymoma, antibodies to ACh-AB
354
What is the tensilon/edrophonium test, and why is it no longer used?
Patients given IV edrophonium chloride, which will reduce muscle weakness temporarily. Rarely done as risk of cardiac arrythmia
355
What is the first line treatment of M.G?
Long acting acetylcholinesterase inhibitors; pyridostigmine is first line
356
What medication is usually given alongside acetylcholinesterase inhibitors in AG?
Immunosuppression; usually prednisolone
357
What is myasthenic crisis?
The potentially life threatening complication of myasthenia gravis which is usually triggered by another illness
358
What is the treatment of myasthenic crisis?
Non-invasive ventilation or mechanical ventilation. Also give IV immunoglobulins and plasmapheresis
359
What is the Barthel index?
A scale that measures disability or dependence in activities of daily living in stroke patients
360
What anti emetic should be prescribed in Parkinsons?
Domperidone: it doesn't cross the blood brain barrier and therefore doesn't exacerbate symptoms of Parkinsons
361
What is the inheritance of Huntingtons?
Autosomal dominant
362
What occurs on a genetic level to cause huntingtons?
Trinucleotide repeat disorder involving a mutation which does for huntington
363
Why is the way that Huntingtons is inherited particularly important?
Displays genetic anticipation, which is a feature of trinucleotide repeat disorders. Leads to earlier age of onset through generations and increased severity of disease
364
What are the first features usually noticed in Huntingtons?
Begins with cognitive, psychiatric or mood problems. General personality changes
365
What is seen in the mood disorder component of huntingtons?
Chorea, dystonia, rigidity, eye movement disorders, dysarthria, dysphagia
366
How is huntingtons diagnosed?
Via genetic testing
367
What is the treatment for huntingtons?
There is no medication that stops/slows the disease
368
What is used to manage chorea in Huntingtons?
Tetrabenazine
369
What is often the cause of death for someone with Huntingtons?
Aspiration pneumonia
370
What is the prognosis for Huntingtons?
Around 20 years
371
What is bulbar palsy?
Signs and symptoms linked to the impaired function of the lower cranial nerves
372
Which nerves are typically affected in bulbar palsy?
9, 10, 11, 12 (the cranial nerves that arise directly from the brainstem)
373
What symptoms are seen in bulbar palsy?
Depends on cranial nerve, but dysphagia, reduced gag reflex, slurred speech, dysphonia and difficulty articulating words (dysarthria), weakness of jaw and facial muscles
374
What is the difference in presentation between bulbar palsy and pseudobulbar palsy?
In pseudobulbar palsy there is changes to emotions, whereas there are no changes in bulbar palsy. There will also be an exaggerated jaw jerk in pseudobulbar palsy
375
What is damaged in pseudobulbar palsy?
Damage to UMN
376
What are the causes of bulbar palsy?
Brainstem strokes and tumours, degenerative diseases (ALS, MND), autoimmune diseases (GBS), genetic conditions
377
What is the inheritance of neurofibromatosis type 1?
Dominant
378
What are 7 features of neurofibromatosis type 1?
1. Cafe au lait spots
2. Relative with RF1
3. Axillary or inguinal freckling
4. Bony dysplasia (presents as bowing of long bone)
5. Iris haemartomas
6. Neurofibromas
7. Glioma of the optic pathway
379
What is seen on eye examination in a patient with neurofibromatosis type 1?
Iris hamartomas (Lisch nodules): yellow brown spots on the iris
380
What are neurofibromas?
Skin coloured, raised nodules with a smooth regular surface
381
How many neurofibromas are required to be significant?
2 or more
382
What is a plexiform neurofibromas?
Large, irregular, complex neurofibroma containing multiple cell types
383
How many plexiform neurofibromas are significant?
More than 1
384
What are the possible complications of neurofibromatosis?
Migraines, epilepsy, renal artery stenosis, learning disability, scoliosis of the spine, vision loss (due to optic nerve gliomas), malignant peripheral nerve sheath tumours, gastrointestinal stromal tumour, brain tumours, spinal cord tumours, increased general risk of cancer
385
What are the possible oncological complications of neurofibromatosis type 1?
Gastrointestinal stromal tumour, malignant peripheral nerve sheath tumours, brain tumour, spinal cord tumours, and just general increased risk of cancer, phaeochromocytoma
386
What might vision loss occur in neurofibromatosis type 1?
Due to optic nerve gliomas
387
What is the pathophysiology of neurofibromatosis type 2?
Schwannomas form (benign tumours of schwann cells) due to mutation in the merlin protein, which is a tumour suppressor protein in Schwann cells
388
What is a common association with neurofibromatosis type 2?
Bilateral acoustic neuromas (tumours of the auditory nerve)
389
What size/frequency of cafe au lait spots are required to be significant?
More than 6, more than 15mm
390
Why are regular blood pressure checks required in neurofibromatosis type 1?
Due to risk of pheochromocytomas
391
What does phaeochromocytomas secrete an excessive amount of?
Catecholamines (adrenaline)
392
Where is adrenaline produced?
In the chromaffin cells in the medulla of the adrenal cells
393
What is the 10% rule in phaeochromocytomas?
10% bilateral, 10% cancerous, 10% outside the adrenal gland
394
What are the presentations of phaeochromocytomas?
Related to excessive adrenaline: anxiety, sweating, headache, tremor, palpitations, HTN, tachycardia
395
Why aren't phaeochromocytomas dx via serum adrenaline?
Due to very short half life, this is not reliable
396
What is the tests performed for phaeochromocytoma?
Plasma free metanephrines, 24-hour urine catecholamines
397
What is the management of phaeochromocytomas?
Alpha blockers, beta blockers, surgical removal of the tumour
398
What causes Wernicke's encephalopathy?
Thiamine deficiency. Commonly in alcoholics, persistent vomiting
399
What is the classic triad of Wernickes?
Ophthalmoplegia/nystagmus, ataxia, encephalopathy
400
What are the features of encephalopathy in Wernicke's?
Confusion, disorientation, indifference, inattentiveness --> personality change
401
What is the pathophysiology of Wernicke's encephalopathy?
Petechial haemorrhages in the brain
402
What is the features of Korsakoff's syndrome?
Wernickes triad + retrograde/anteretrograde amnesia, and confabulation
403
What should the loss of corneal reflex make you consider?
Acoustic neuroma
404
What is the cushing reflex?
A physiological nervous system response to increased intracranial pressure that results in HTN and bradycardia
405
What is the largest risk factor for dementia?
Increasing age
406
How can dementia only be definitively diagnosed?
Post mortem
407
What manner inheritance is shown in Alzheimer's disease (for those who have a genetic component)?
Autosomal dominant
408
What are risk factors for Alzheimer's disease?
Increasing age, FHx, genetics, caucasian, Down's syndrome
409
What is the macroscopic changes seen in Alzheimer's?
Widespread cerebral atrophy, particularly involving the cortex and hippocampus
410
What is the microscopic pathological changes seen in Alzheimers? How does this lead to disease?
Plaques due to deposition of beta-amylois protein, and intraneuronal neurofibrillary tangles due to abnormal aggregation of tau protein. This leads to reduction in transmissions in the brain, and death of cells
411
What is the first line pharmocological treatment of Alzheimers?
Acetylcholinesterase inhibitors (donepezil, galantamine, rivastigmine)
412
What is the second line treatment for Alzheimer's?
Memantine
413
When is donepezil used, and what is it's contraindication?
First line tx of Alzheimers. C/I in patients with bradycardia
414
What is the adverse effects of donepezil?
Insomnia
415
Should antidepressants be used in patients with mild/moderate depression with dementia?
No
416
What are levels of ACh usually reported as in patients with Alzheimers?
Reduced
417
What is the most common presentation of Alzheimers?
Memory loss, particularly recent. Associated changes in planning, reasoning, speech and orientation
418
What is memory loss is depression like in comparison to dementia?
Global memory loss in depression, recent memory loss in dementia
419
What are the risk factors of vascular dementia?
Hx of stroke/TIA, AF, HTN, DM, hyperlipidaemia, smoking, obesity, CHD
420
How do patients with vascular dementia typically present?
Stepwise deterioration of cognitive impairment
421
What are typical features of vascular dementia?
Focal neurological abnormalities (visual disturbance, sensory, motor symptoms), difficulty with attention/concentration, seizures, memory + speech + gait disturbance, mood disturbance and disorders
422
How is vascular dementia diagnosed?
MRI can show infarcts and extensive white matter changes. Can also do CT
423
What areas of the brain are most affected by vascular dementia?
White matter of hemispheres, grey nuclei, thalamus, striatum
424
What is the management of vascular dementia?
Generally symptomatic, and reducing cardiovascular risk factors to reduce progression. No use of AChE inhibitors or memantine is indicated
425
What type of dementia is associated with MND?
Frontotemporal dementia (Pick's disease)
426
What is the pathophysiology of Lewy Body dementia?
Alpha-synuclein cytoplasmic inclusions (Lewy bodies) in the substantia nigra, paralimbic and neocortical areas
427
What disease is strongly associated with Lewy body dementia?
Parkinsons
428
What are the features of LewyBody dementia?
Progressive cognitive impairment comes first, then parkinsonism (triad), visual hallucinations
429
What is unique about cognition impairment in Lewy Body dementia in comparison to other dementias?
Cognition may be fluctuating, which is unique
430
What is the diagnosis of a patient with visual hallucinations and dementia?
LewyBody dementia
431
How does Lewy Body dementia typically present?
Problems multitasking and performing complex cognitive actions (rather than memory)
432
What dementia classical affects younger people?
Frontotemporal dementia (including Pick's disease)
433
What are the three recognised types of frontotemporal lobar degeneration?
- frontotemporal dementia (Pick's disease)
- semantic dementia
- progressive non fluent aphasia
434
What are the common features of frontotemporal lobar dementias?
Onset before 65 years, insidious onset, relatively preserved memory and visuospatial skills, personality change and social conduct problems
435
How does Pick's disease typically present?
Personality change, impaired social conduct, disinhibition, increased appetite
436
What is seen on imaging in Pick's disease?
Focal gyral atrophy with a knife blade appearance
437
What is the microscopic changes in Pick's disease?
- Pick bodies (spherical aggregations of tau protein)
- neurofibrillary tangles
- plaques
438
What are the features of progressive non fluent aphasia (type of frontotemporal lobar degeneration)
Non fluent speech, issues with grammar and literacy skills
439
What category of dementias does semantic dementia fall under?
Frontotemporal lobar degeneration
440
What is the presentation of semantic dementia?
Decline in understanding of word meaning, issues with word retrieving. Empty speech with no meaning. Memory better for recent events than remote ones
441
What are differential dx for dementia?
Prion protein diseases (CJD), HIV dementia, normal pressure hydrocephalus, severe depression, mild cognitive impairment, late syphilis
442
What is Creutzfeldt-Jakob disease?
Rapidly progressive neurological condition caused by prion proteins. Causes the brain to fold abnormally. Sporadic or variant
443
What is the aetiology of variant CJD?
Caused by meat infected by bovine spongiform encephalopathy
444
What is the disease course of CJD?
Disease can be indolent for many years. Then presents as minor memory lapses, mood disturbance, loss of interest. Quickly becomes more prominent, with unsteadiness, stiffness, jerking movements, incontinence. Death occurs within 6 months.
445
What is normal pressure hydrocephalus?
Secondary to reduced CSF absorption at the arachnoid villi
446
What is the common causes of normal pressure hydrocephalus?
Head injury, SAH, meningitis
447
What is the classic triad of normal pressure hydrocephalus?
Urinary incontinence, dementia, gait abnormality (wet, wacky, wobbly)
448
What is seen on imaging of normal pressure hydrocephalus?
Ventriculomegaly in the absence of, or out of proportion, to sulcal enlargement
449
What is the treatment of normal pressure hydrocephalus?
VP shunt: can reverse the condition
450
Where is CSF produced, and where is it absorbed?
CSF is created in the four choroid plexuses (one in each ventricle) and by the walls of the ventricles. CSF is absorbed into the venous system by the arachnoid granulations.
451
What is the most common cause of paediatric hydrocephalus?
Aqueductal stenosis, leading to insufficient drainage of CSF
452
What are possible congenital causes of paediatric hydrocephalus?
Arachnoid cysts, arnold-Chiari malformation, chromosomal abnormalities
453
What is arnold chiari malformation?
Where the cerebellum herniates downwards through the foramen magnum, blocking CSF outflow
454
How to children with hydrocephalus typically present?
Rapidly enlarging head circumference, bulging anterior fontanelle, poor feeding and vomiting, poor tone, sleepiness
455
What are possible VP shunt complications?
Infection, blockage, excessive drainage, intraventricular haemorrhage during shunt related surgery
456
What factors suggest depression over dementia?
Short history, rapid onset, biology symptoms (weight loss, sleep disturbance), patient worried about poor memory, reluctant to take tests + dissappointed with results, variable MMSE results, global memory loss
457
How are MMSE results interpreted?
Any score of 24 or more indicates a normal cognition. Below this can indicate Alzheimers
458
What is mild cognitive impairment?
Term used for those with memory problems or higher cortical thinking, but not severe enough to interfere with everyday life
459
Why might AF and anaemia worsen cognitive impairment?
Leads to mild brain hypoxia
460
What is pellagra? What causes it
Deficiency disease of vitamin niacin (B3)
461
What groups of people are more at risk of pellagra?
Those having isoniazid therapy (TB), and alcoholics
461
What are the classical symptoms of Pellagra?
Dermatitis, diarrhoea, dementia, death if not treated
462
What are possible reversible causes of dementia?
Subdural haematoma, normal pressure hydrocephalus, vitamin deficiency, hypothyroidism
463
What factors indicate a diagnosis of delirium is more likely than dementia?
Acute onset, impairment of consciousness, fluctuation of symptoms (worse at night), abnormal perceptions (hallucinations), agitation, fear, delusions, inattention
464
What factors increase risk of delirium?
>65 years, hx of dementia, significant injury (eg, hip fracture), frailty or multimorbidity, polypharmacy
465
What is delirium?
Acute confusional state that causes disturbed consciousness, attention, congition and perception
466
What are possible causes of delirium?
Infection (particularly UTI), metabolic (hypercalcaemia, hypoglycaemia, hyperglycaemia, dehydration), change of environment, severe pain, alcohol withdrawal, constipation, hypothermia, organ dysfunction, new medications, nutrition changes
467
What are the features of delirium?
Memory disturbances (loss of short term), agitation, withdrawn, disorientation, mood change, visual hallucinations, disturbed sleep, poor attention
468
What are the three types of delirium?
Hypoactive, hyperactive, mixed
469
What is the presentation of hyperactive delirium?
Agitation, delusions, hallucinations, wandering, aggression
470
What are the symptoms of hypoactive delirium?
Lethargy, slowness w/ everyday tasks, excessive sleeping, inattention
471
What is the management of delirium?
Treat the cause. First line medication is haliperidol
472
What is the first line medication in delirium?
Haloperidol
473
What cognitive tests can be used in delirium?
CAM (confusion assessment method), 4A's test, AMT (abbreviated mental test)
474
What is the investigations in delirium?
ECG, cultures, FBC, U+Es, LFTs, calcium, HbA1c, CRP, drug levels, syphilis serology, CXR, CT head
475
What is Sundowner syndrome in dementia?
As evening approaches confusion increases and there is more falls
476
What is the mechanism that causes the presentations of alcohol withdrawal?
Chronic alcohol consumption enhances GABA mediation and inhibits glutamates. Thus opposite in withdrawal (less GABA, more glutamate)
477
When do features of alcohol withdrawal begin?
6-12 hours
478
What features are seen at 6-12 hours after alcohol withdrawal?
Tremor, sweating, tachycardia, anxiety, nausea and vomiting
479
What symptom is at peak incidence at 36 hours after alcohol withdrawal?
seizures
480
What is the peak incidence of delirium tremens after alcohol withdrawal?
48-72 hours
481
What symptoms are seen in delirium tremens?
Coarse tremor, confusion, delusions, auditory and visual hallucinations, fever, tachycardia, autonomic hyperactivity
482
What is first line in management of alcohol withdrawal?
Long acting benzo: chlordiazepoxide or diazepam
483
What is the treatment of Wernicke's?
Pabrinex IV (vitamin B and C), and thiamine
484
What vessel sizes are affected in temporal arteritis?
Medium and large sized vessels
485
What condition has a strong link with giant cell arteritis?
Polymyalgia rheumatica
486
What are the features of polymyalgia rheumatica?
Rapid onset acheing morning stiffness in proximal limb muscles (girdle) with no weakness. Also lethargy, depression, low grade fever, anorexia, night sweats
487
What is the treatment of polymyalgia rheumatica?
Prednisolone: if no response, consider alternative diagnosis
488
What is seen on investigation of polymyalgia rehumatica?
Raised inflammatory markers, normal creatine kinase
489
What is the main complication of giant cell arteritis?
Irreversible vision loss
490
What age is a typical patient with giant cell arteritis?
>60 years old
491
What are the features of giant cell artertis?
Unilateral headache (severe, around temple and jaw), scalp tenderness, jaw claudication, blurred or double vision, amaurosis fugax (though may become permanent)
492
What might be noticed on examination of the face in giant cell arteritis?
Temple artery may be tender and thickened to palpate, with reduce/absent pulsation
493
What is a key testing in all patients with giant cell arteritis?
Vision testing
494
Why would diplopia occur in giant cell arteritis?
If there is involvement of the cranial nerves
495
What is the main ocular complication in giant cell arteritis?
Anterior ischaemic optic neuropathy (leads to ischaemic of the optic nerve head)
496
What is seen on fundoscopy of giant cell arteritis?
Anterior ischaemic optic neuropathy; swollen pale disc and blurred margins
497
What investigations are done in giant cell arteritis?
Raised inflammatory markers, temporal artery biopsy, ultrasound of temporal artery
498
What is seen on duplex ultrasound in giant cell arteritis?
Halo sign, and stenosis of the temporal artery
499
What is the immediate treatment of GCA?
High dose steroids: no visual loss then high dose prednisolone, if evolving visual loss then methylprednisolone first then prednisolone
500
When should steroids be given in GCA?
Immediately: before temporal artery biopsy
501
What is long term management in GCA patients?
- bone protection with bisphosphonates due to long course of steroids required
- low dose aspirin
- PPI for gastroprotection on steroids
502
What is the diagnosis criteria for chronic fatigue syndrome?
Disabling fatigue for 3 months, for more than 50% of time, affecting mental and physical function in absence of other disease which could explain symptoms
503
What are the features of chronic fatigue syndrome?
Fatigue made worse by activity and not relieved by rest, sleep problems (insomnia, hypersomnia, unrefreshing sleep), muslce/joint pains, headaches, painful lymph nodes, sore throat, cognitive dysfunction, flu like symptoms, dizziness, nausea, palpitations
504
How long must symptoms be present for chronic fatigue syndrome to be diagnosed?
3 months
505
What investigations should be carried out for chronic fatigue syndrome?
Large numbers of screening blood tests to exclude other pathology (eg, FBC, U&E, LFT, glucose, TFT, ESR, CRP, calcium, CK, ferritin, coeliac screen)
506
What is the management for chronic fatigue syndrome?
- refer to specialist service
- energy management (spoons theory)
- only physical exercise where possible for them
- CBT
507
What are the features of Horner's syndrome?
Miosis (small pupil), ptosis, enopthalmos (sunken eye), anhidrosis (loss of sweating on one side)
508
Where does head/arm/trunk anhidrosis suggest the lesion is and what has potentially caused it?
Central lesion. Caused by Stroke, spingomyelia, MS, brain tumours
509
Where is the lesion if there is anhidrosis of only the face? What are possible causes?
Pre-ganglionic lesion. Pancosts tumour (apical lung cancer), trauma, thyroidectomy
510
Where is the lesion if there is Horner's syndrome without anhidrosis? What could cause this?
Post ganglionic. In carotid artery (dissection) or cavernous sinus thrombosis
511
What causes Horner's syndrome?
Lesion of the sympathetic chain supplying the eye
512
What structures does the sympathetic nervous system supply in the eye?
Dilator pupillae (dilation of the pupil), sweat gland, muscle response for raising upper eyelid
513
How is Horner's syndrome classified?
Via the site of the lesion in the sympathetic chain. There are three consecutive neurons which transmit signals from the hypothalamus to the eye
514
What type of Stroke does Central Horner's typically present with?
Lateral medullary syndrome (posterior inferior cerebellar artery) --> ipsilateral ataxia, dysphagia, ipsilateral facial pain and temperatre loss, nystagmus
515
Where does the first order neurone of the sympathetic chain run between?
Hypothalamus and T1 spinal cord
516
Where does the second order neurone of the sympathetic chain run between?
T1 spinal cord to the superior cervical ganglion. Goes near to the upper lobe of the lung and subclavian artery
517
Where does the third order neurone of the sympathetic chain run between?
From superior cervical ganglion to the cavernous sinus, where sympathetic fibres join the ophthalmic nerve
518
What is a classic finding in congenital Horner's syndrome?
Iris heterochromia
519
How does Pancoast's tumour present?
Horners syndrome with anhidrosis of the face, shoulder pain, upper limb neurological symptoms
520
How is Horner's diagnosed (if clinical signs unclear)
Eye drops: apraclonidine which will reverse pupillary constriction in Horners, but not in a normal pupil
521
How is Horners syndrome treated?
By treating the cause!
522
How is neuropathic pain treated?
Amitriptyline, duloxetine, gabapentin, pregabalin
523
How does diabetic neuropathy typically present?
Sensory loss, not motor. This is typically in a glove and stocking distribution. Lower lengths affected first
524
What can be used as rescue therapy in exacerbations of neuropathic pain?
Tramadol
525
How is diabetic neuropathy managed?
The same way as other forms of neuropathic pain (amitriptyline, duloxetine, gabapentin, pregabalin)
526
What should be used for localised neuropathic pain?
Topical capsaicin
527
What two types of neuropathy can occur in diabetes?
Peripheral neuropathy and gastrointestinal autonomic neuropathy
528
How does gastrointestinal autonomic neuropathy present?
Gastroparesis, chronic diarrhoea, GORD
529
What are the symptoms of gastroparesis?
Erratic blood glucose control, bloating, vomiting
530
How is gastroparesis managed?
Metoclopramide, domperidone, erythromycin
531
What are causes of predominantly motor loss peripheral neuropathy?
GBS, lead poisoning, Charcot Marie Tooth, diphtheria
532
What causes predominantly sensory loss peripheral neuropathy?
Diabetes, uraemia, leprosy, alcoholism, B12 deficiency, amyloidosis
533
What occurs in vitamin B12 deficiency that leads to peripheral neuropathy?
Subacute combined degeneration of the spinal cord. Dorsal column affected first
534
What does the dorsal column convey?
Fine touch vibration, two point discrimination, proprioception
535
What does the spinothalamic tract convey?
Pain, thermal stimuli, pressure, crude touch
536
What is Phalen's test?
Assess Carpal tunnel. Hold wrist in maximum flexion, and if the test is positive there is numbness in the median nerve distribution
537
What type of headache occurs in migraine?
Severe, unilateral, throbbing, aggravated by physical activity
538
How do migraines present in children?
Can be bilateral, more commonly also have GI disturbance
539
How long do migraine attacks last?
Up to 72 hours
540
What sort of aura is seen in migraine?
Visual, progressive, last 5-60 minutes
541
What gender are migraines more common in?
Women
542
What are common triggers for migraines?
Tiredness, stress, alcohol, COC, lack of food or dehydration, cheese, chocolate, red wine, menstruation, bright lights
543
How many migraine attacks must have occurred for a diagnosis to be made?
At least 5
544
What is a hemiplegic migraine?
A variant of migraine in which motor weakness is a manifestation of aura
545
What percentage of migraines present with an aura?
25%
546
What are atypical aura symptoms that should be investigated?
Motor weakness, double vision, visual symptoms affecting only one eye, poor balance, decreased level of consciousness
547
What is the general rule of migraine management in terms of drug classes?
5-HT agonists are used in acute treatment, 5-HT receptor antagonists are used in prophylaxis
548
What is first line treatment in acute migraine attack?
Oral triptan + NSAID, or oral triptan and paracetamol
549
What can be added onto acute first line migraine treatment?
Anti emetics: metoclopramide, domperisone, prochloperazine
550
Why should metoclopramide not be prescribed to young people?
Increased risk of acute dystonic reaction
551
When should migraine prophylaxis be given?
When there is significant impact on quality of life, and occur frequently
552
What is the first line migraine prophylaxis?
Propanolol or topiramate (teratogenic!) or amitriptyline
553
What vitamin medication may reduce migraines?
Riboflavin (Vit B2)
554
What is the treatment for women with predictable menstrual migraine treatment?
Frovatriptan or zolmitriptan
555
What is first line medication of migraine in pregnancy?
Paracetamol
556
What is second line management of migraine in pregnancy?
NSAIDs (only 1st and 2nd trimester)
557
Why can some migraine patients not have the COC?
If migraine with aura, due to increased risk of stroke
558
Can patients with history of migraines be prescribed HRT?
Yes, but may make migraines worse
559
What are the four types of migraine?
With aura, without aura, silent (with aura but no headache), hemiplegic
560
What are the 5 stages of migraine?
Prodromal, aura, headache, resolution, post dromal
561
What is the strongest risk factor for hemiplegic migraine?
Family history: autosomal dominant condition
562
What is the drug class of triptans?
5-HT receptor agonists
563
What is the MoA of triptans?
Cranial vasoconstriction, inhibition of transmission of pain signals, inhibiting the release of inflammatory neuropeptides
564
How many doses of triptans can be taken in migraine?
2 (Additional one taken if the first doesn't work)
565
What are contraindications for triptans?
Being on SSRIs, HTN, coronary artery disease, previous stroke, TIA (aka risks related to vasoconstriction)
566
What is a tension headache?
Recurrent, bilateral headache, non-disabling, often described as a 'tight band'
567
Who are cluster headaches more common in?
Men and smokers
568
What is the frequency and duration of cluster headaches?
Once or twice a day, episodes lasting 15 mins -2 hours, clusters lasting 4-12 weeks
569
What are the symptoms of cluster headaches?
Intense pain around eye (always same side), patient restless during attack, accompanied by redness, lacrimation, lid swelling
570
What is the typical presentation of medication overuse headache?
Present for 15 days or more per month, despite using regular sumitriptan
571
What are possible causes of acute single episode of headache?
Meningitis, encephalitis, SAH, head injury, sinusitis, glaucoma (acute closed angle), tropical illness (eg, malaria)
572
What are possible causes of chronic headache?
Chronically raised ICP, Paget's disease, psychological
573
What is the most common cause of primary headache in children?
Migraine without aura
574
What are the side effects of triptans?
Tingling, heat, heaviness/pressure sensations
575
What is the first line in children for migraine?
Ibuprofen (more effective than paracetamol in kids)
576
When can triptans be used in children, and what type is preferred?
If over 12, and oral spray is preferred
577
What is the second most common cause of headache in children?
Tension type headache
578
What are red flags for headaches?
Immunocompromised, under 20 + hx of malignancy, hx of malignancy to brain, vomiting without other obvious cause, w/ fever, thunderclap, new onset neurological deficit, new onset cognitive dysfunction, change in personality, impaired level of consciousness, recent head trauma, triggered by cough/sneeze/valsalva , changes with posture, change in character of headache
579
What are sinister signs of headaches that require urgent head CT?
Cognitive dysfunction, impaired level of consiousness, thunderclap
580
What are the features of post lumbar puncture headache?
Develops a day after LP, lasts several days, worsens when upright, improves lying down
581
Who are post LP headaches more common in?
Women with low BMI
582
What are factors that contribute to potential post LP headache?
Increased needle size, direction of bevel, increased number of LP attempts
583
Does opening pressure + volume of CSF removed in an LP affect the change of a post LP headache?
Nope
584
What is the treatment for post LP headache?
Initially supportive, eventually may need epidural saline and IV caffeine
585
What can trigger an attack of cluster headaches?
Alcohol
586
What is the investigation of choice in cluster headaches?
MRI with contrast (sometimes there are underlying brain lesions)
587
What is the acute treatment of cluster headaches?
100% oxygen, subcut triptan
588
What is the prophylaxis of cluster headaches?
Verapamil
589
What can differentiate a tension headache from a migraine?
Bilateral, lower intensity, no aura or N+V or aggravation by physical activity
590
What is the definition of a chronic tension type headache?
Present on 15 or more days per month
591
What is the acute treatment of tension type headache?
aspirin, paracetamol or NSAID
592
What is prophylaxis of tension headache?
NOT TRIPTANS! Course or acupuncture
593
Which patients are most at risk of medication overuse headache?
Patients using opioids or triptans
594
What is the management of medication overuse headache?
Withdraw simple analgesic and triptans abruptly. Opioid analgesic withdrawn gradually
595
What investigations can be done in migraine to identify red flag causes? (not radiological)
Fundoscopy for papilloedema
596
Why is it worrying if ipsilateral signs start in a patient who has had a cerebrovascular event?
Midline has occurred and the other side has started to be affected
597
Why is it worrying if a pupil is blown in a patient who has had a cerebrovascular event?
Means that the brain is starting to cone (herniation), as it is compressing the 3rd nerve nucleus in the midbrain
598
What side is most people's language centres located?
Left
599
When are hormonal headaches more likely to occur?
Two days before or 3 days after the menstrual period, in the perimenopausal period, early pregnancy
600
What is cervical spondylosis and how does it typically present?
Common condition that results from OA. Presents as neck pain that may be referred and mimic headaches
601
What are possible complications of cervical spondylosis?
Radiculopathy and myelopathy
602
What is radiculopathy?
Pinched nerve: an injury or damage to nerve roots in the area where they leave the spine
603
What is myelopathy?
An injury to the spinal cord caused by severe compression that may be due to spinal stenosis, disc degeneration, disc herniation, AI disorders, trauma
604
What causes brain abscess?
Extension of sepsis from middle ear or sinuses, trauma or surgery to the scalp, penetrating head injuries, embolic events from endocarditis
605
What is the presentation of brain abscess?
Depends on the site. Also: headache, fever, focal neurology. Symptoms of raised intracranial pressure (nausea, papilloedema, seizures)
606
What is the primary infection of a suspected brain abscess? How does it present?
MRI: ring enhancing lesion with surrounding oedema
607
What is the management of brain abscess?
Surgery (craniotomy + drainage), IV abx (IV ceft/cefotaxime + metronidazole), intracranial pressure management (eg, dexamethasone)
608
What is normal ICP?
7-15
609
What is cerebral perfusion pressure and how is it calculated?
Net pressure gradient causing cerebral blood flow to the brain. CPP=mean arterial pressure-ICP
610
What causes raised ICP?
Idiopathic intracranial HTN, traumatic head injury, infection (meningitis, abscess), tumours, hydrocephalus
611
What is Cushing's triad?
Widening pulse pressure, bradycardia, irregular breathing
612
How is raised ICP investigated/monitored?
Neuroimaging, invasive ICP monitoring
613
What is the cutoff for determining if further treatment is needed to reduce ICP?
>20 mmHg
614
What is the management of raised ICP?
Head elevation to 30 degrees, IV mannitol, controlled hyperventilation, removal of CSF (shunt/drain/LPs in idiopathic intracranial HTN)
615
When is controlled hyperventilation used?
In raised ICP
616
How is controlled hyperventilation helpful in raised ICP?
Reduces pCO2 which leads to vasoconstriction of cerebral arteries, which reduces ICP
617
What are risk factors for idiopathic intracranial HTN?
Obesity, female, pregnancy, certain drugs
618
What drugs can cause Idiopathic intracranial HTN?
COCP, steroids, tetracyclines (doxycycline), lithium
619
What cranial nerve is most likely to be affected by idiopathic intracranial HTN?
6th (abducens) (because of its long course)
620
What are the features of idiopathic intracranial hypertension?
Headache, blurred vision, papilloedema, enlarged blind spot, 6th nerve palsy
621
What is the lifestyle management of idiopathic intracranial HTN?
Weight loss
622
What is the medical/surgical management of idiopathic intracranial hypertension?
Carbonic anhydrase inhibitors (acetazolamide), as they reduced CSF production at the choroid plexus. Topiramate also inhibits it. Can do repeated lumbar puncture
623
What is GBS?
Acute paralytic polyneuropathy affecting the PNS
624
What are common causative organisms of GBS?
Campylobacter jejuni, CMV, EBV
625
What is the pathophysiology of GBS?
B cells create antibodies against the antigens on the triggering pathogens, which also match the proteins of the peripheral neurones. They target the myelin sheath or nerve axon itself
626
What antibodies are typically found in GBS?
anti-GM1 antibodies (anti ganglioside)
627
What is Miller Fisher syndrome?
Variant of Guillain Barre syndrome: descending paralysis. Eyes are typically affected first, also ataxia.
628
What is the most common symptom seen in initial stages of illness in GBS?
Back/leg pain
629
What are the characteristic features of Guillain Barre syndrome?
Progressive, symmetrical weakness of all limbs, ascending. Reduced/absent reflexes. Few sensory signs
630
What is the differential diagnosis if there is symmetrical limb weakness following gastroenteritis?
GBS
631
What are later stage symptoms of GBS?
respiratory muscle weakness, cranial nerve involvement
632
What is the first line investigation in GBS? What also may be done?
Lumbar puncture: rise in protein with normal WBC. Also, nerve conduction studies which will show decreased motor nerve conduction velocity
633
What is first line management of GBS?
Iv immunoglobulins
634
What is the leading cause of death in GBS and thus what prophylaxis should be given?
Pulmonary embolism is leading cause. VTE prophylaxis should be given
635
Does unilateral cerebellar lesions cause ipsilateral or contralateral lesions?
Ipsilateral
636
What are the symptoms of cerebellar disease?
DANISH. Dysdiadochokinesia/dysmetria (past pointing), ataxia (limb, truncal), nystagmus (horizontal), intention tremour, slurred speech, hypotonia
637
What are causes of cerebellar syndrome?
Friedreich's ataxia, neoplasms (cerebellar haemangioma), stroke, alcohol, MS, hypothyroidism, brain mets (eg, secondary to lung cancer)
638
What is Friedreich's ataxia?
Early onset ataxia
639
What is the inheritance of Friedreich's ataxia?
Autosomal recessive
640
How does Friedreich's ataxia present?
Gait ataxia and kyphoscoliosis in young teenagers
641
What are the neurological signs Friedreich's ataxia?
Absent ankle jerks, cerebellar ataxia, optic atrophy, spinocerebellar tract degeneration
642
What is the most common cause of death in Friedreich's ataxia?
Hypertrophic obstructive cardiomyopathy
643
What are the features of encephalitis?
Fever, headache, psychiatric symptoms, seizures, vomiting, focal features
644
What causes encephalitis most commonly?
Herpes zoster virus 1
645
What areas of the brain are most likely to be affected by encephalitis?
Temporal and inferior frontal lobes
646
What are the investigations of encephalitis?
LP: high white cells, elevated proteins, and do PCR for HSV/VZV. Neuroimaging (MRI), and EEG
647
What is the management of suspected encephalitis?
IV aciclovir: start immediately
648
What is first line imaging in encephalitis?
MRI
649
What characteristically causes temporal lobe encephalitis?
Herpes simplex
650
How does primary infection of herpes simplex virus often present?
Severe gingivostomatitis (infection of the mouth and gums, with swelling)
651
What causes malaria?
Plasmodium protozoa. There are four types, but plasmodium falciparum causes nearly all episodes, and plasmodium causes some
652
What are protective factors against malaria?
G6PD deficiency, HLA-B53
653
What are the features of severe malaria?
Schizonts on a blood film, parasitaemia >2%, hypoglycaemia, acidosis, pyrexia, severe anaemia, experiencing complications
654
What are possible complications of malaria?
Cerebral malaria (seizures, coma), acute renal failure, acute respiratory distress syndrome, hypoglycaemia, DIC
655
What are first line treatment of uncomplicated malaria?
Artemisinin-based combination therapies. Usually artemether with lumefantrine
656
What vector spreads malaria?
Female anopheles mosquitos
657
What is the presentation of malaria?
Unspecific: fever (very high and spikes at 48 hours), fatigue, myalgia, headache, nausea, vomiting
658
What signs are seen on examination in malaria?
Pallor due to anaemia, hepatosplenomegaly, jaundice
659
How is malaria diagnosed, and how is it proven to be negative?
Malaria blood film (shows parasite, concentration, and type). Three negative samples needed over 3 consecutive days due to parasites being released from red blood cells into the blood every 48-72 hours
660
What is the treatment for severe or complicated malaria?
Artesunate
661
What are acoustic neuromas?
Vestibular Schwannomas: a cerebellopontine angle tumour
662
What is the classical history of vestibular schwannoma?
Combination of vertigo, hearing loss, tinnitus, absent corneal reflex
663
What cranial nerves are involved in Vestibular schwannoma?
5, 7, 8,
664
What is an important differential in unilateral hearing loss or tinnitus?
Vestibular schwannoma
665
What is the first line investigation for vestibular schwannoma?
MRI of the cerebellopontine angle. Also audiometry
666
What are the symptoms of spinal metastases?
Unrelenting lumbar back pain, any thoracic or cervical back pain, worse with coughing/sneezing, nocturnal, associated with tenderness. Later: neurological features
667
What is the most common form of brain cancer?
Metastatic
668
What are the 2 important features of brain mets?
There are often multiple, and often not treatable with surgical intervention
669
What tumour most commonly spread to the brain?
Lung, breast, bowel, skin (melanoma), kidney
670
What tumour most commonly spreads to the brain?
Lung
671
What is gliobastoma multiforme? What is their unique feature?
Highly malignant and invasive brain tumour which crosses the corpus callosum (midline)
672
What is the most common primary brain tumour in adults?
GMB (glioblastoma multiforme)
673
How are GBMs treated?
Surgery with postoperative chemo and/or radiotherapy. Dexamethasone to treat oedema
674
How do GBMs appear on imaging?
Solid tumours with central necrosis and rim that enhances with contrast
675
What is olgiodendroma? How do they appear on histology?
Benign and slow growing tumour. On histology: fried egg appearance
676
What are meningiomas?
Benign, superficial (extrinsic), arachnoid origin
677
What are the symptoms of meningiomas?
Compression rather than invasion: can cause very late onset of symptoms!
678
How do meningiomas present on histology?
Spindle cells + psammoma bodies?
679
What is haemangioblastoma, and what syndrome is it associated with?
Vascular tumour of the cerebellum, can produce EPO. Associated with von Hippel-Lindau syndrome (syndrome that causes haemangioblastomas of multiple locations)
680
What are the two types of pituitary adenoma?
Secretory or non secretory
681
What are the common presentations of pituitary adenoma?
Bitemporal hemianopia due compression of optic chiasm (due to closeness) due to mass effect. Also consequences of hormone excess. Headache
682
What surgery is done in pituitary adenoma?
Transphenoidal resection
683
What is the most common brain tumour in children?
Pilocytic astrocytoma
684
What brain tumour is a remnants of Rathke's pouch?
Craniopharyngioma
685
What is cerebral palsy?
The disorder of movement and posture due to a non progressive lesion of the motor pathways of the developing brain
686
What are the antenatal causes of cerebral palsy?
Maternal infections, trauma around pregnancy
687
What are the perinatal causes of cerebral palsy?
Birth asphyxia, pre-term birth
688
What are postnatal causes of cerebral palsy?
Meningitis, severe neonatal jaundice, head injury
689
What are the 4 types of cerebral palsy?
Spastic, dyskinetic, ataxic, mixed
690
What causes spastic cerebral palsy, and what are the features?
Damage to UMN. Hypertonia and reduced function.
691
What causes dyskinetic cerebral palsy, and what are the features?
Damage to the basal ganglia. Presents as issues controlling muscle tone, with hypertonia and hypotonia. Causes athetosis (slow, writhing movements of distal extremities), and oro-motor problems
692
What causes ataxia cerebral palsy, and what are the features?
Cerebellum. Issues with coordinating movement
693
694
What are the four patterns of spastic cerebral palsy?
Monoplegia, hemiplegia, diplegia, quadraplegia
695
What are signs and symptoms of cerebral palsy?
Failure to meet milestones, increased or decreased tone, hand preference before 18 months, problems with coordination/speech/walking, feeding or swallowing problems, learning difficulties
696
What gait indicates an upper motor neurone lesion?
Hemiplegic/diplegic gait (gait where the leg is held stiffly and abducted with each step and swung around to the ground infront).
697
What gait indicates a cerebellar lesion?
Broad based/ataxic gait
698
What does a high stepping gait indicate?
Foot drop of LMN
699
What does waddling gait suggest?
Pelvic muscle weakness due to myopathy
700
What does gait in cerebral palsy present as?
Diplegic gait. Abnormally narrow base, dragging both legs and scrapping toes. Can have some circumduction. Arms will likely be flexed
701
What are complications and associated conditions with cerebral palsy?
Learning disability, epilepsy, kyphoscoliosis, muscle contractures, hearing and visual impairment, GORD
702
What is the treatment of spasticity in children with cerebral palsy?
Oral diazepam, baclofen, surgery (release tendons)
703
What treatment might be offered to neonates with hypoxic ischaemic encepholopathy?
Therapeutic cooling: reduces neuronal damage
704
What causes hypoxic ischaemic encepholopathy?
Hypoxic perinatal brain injury: caused by a decrease in the amount of oxygen supplied to infant's brain during labour
705
What is the pathophysiology of hypoxic brain birth injuries?
Biphasic model of neuronal death: death at time of insults and secondary death. Therapeutic cooling can reduce extent of secondary neuronal death
706
When is hypoxic ischaemic encepholopathy in neonates?
Acidosis on umbilical artery blood gas, poor apgar scores, multi organ failure, seizures
707
What are examples of causes of hypoxic-ischaemic encepholopathy?
Maternal shock, intrapartum haemorrhage, prolapsed cord, nuchal cord
708
How is temperature monitored in therapeutic hypothermia?
A rectal probe
709
What is the target temperature of neonatal cooling, and for how long?
33 to 35 degrees celsius. Done for 72 hours
710
What time must therapeutic cooling be initiated in?
6 hours
711
What is the most common area for spinal stenosis?
Lumbar spine
712
What are the causes of spinal stenosis?
Congenitla, degenerative changes, herniated discs, thickening of ligaments in spine, fracture, tumours
713
What is the typical presentation of spinal stenosis?
Gradual onset. Back pain, neuropathic pain, symptoms mimicking claudication (worse on movement, better on rest)
714
What relieves spinal stenosis pain? What condition does this differentiate it from?
Sitting forward and walking uphill (expands the spinal canal). Differentiates this from claudication.
715
What are the symptoms of intermittent neurogenic claudication that are seen in lumbar spinal stenosis?
Lower back pain, buttock and leg pain, leg weakness. Numbness. Better at rest, occur when moving.
716
How does lateral spinal stenosis and foramina stenosis of the lumbar spine present?
Sciatica
717
What is a key differential for central lumbar stenosis?
Peripheral arterial disease: however, there will be normal peripheral pulses and ABI, and presence of back pain
718
What is the first line investigation for spinal stenosis?
MRI
719
What is the surgical management of spinal stenosis?
Laminectomy
720
What are red flags for lower back pain?
<20 years old or >50 years old, hx of previous malignancy, night pain, history of trauma, systemically unwell
721
What is the typical presentation of ankylosing spondylitis?
Young man with lower back pain and stiffness, which is worse in the morning and improves with activity
722
What are possible causes of foot drop?
Common peroneal nerve lesion, L5 radiculopathy, sciatic nerve lesion
723
What is often the cause of common peroneal nerve lesion?
Compression at the neck of the fibula
724
What are precipitating factors of common peroneal nerve lesion?
Prolonged confinement, recent weight loss, Baker's cysts, plaster casts
725
What symptoms suggest L5 radiculopathy?
Weakness of hip adbuction, foot drop
726
What are the symptoms of Carpal Tunnel syndrome?
Pain/pins and needles in the thumb, index, middle finger. Symptoms ascend.
727
What is seen on examination in carpal tunnel syndrome?
Weakness of thumb abduction, wasting of thenar eminence, tinel's sign, phalen's sign
728
What are the causes of carpal tunnel syndrome?
Idiopathic, pregnancy, oedema, rheumatoid arthritis (often bilateral), acromgealy, diabetes, repetitive strain
729
How does carpal tunnel syndrome affect action potentials?
Prolongs motor and sensory action potentials
730
What is the treatment of carpal tunnel syndrome?
6 week of conservative treatments: corticosteroid injection, wrist splints at night. If severe: surgical decompression
731
What reflexes would be reduced with L3/4 radiculopathy?
Knee reflex
732
How can nerve root pain be identified?
Dermatomal distribution, associated neurological signs
733
How can S1 radiculopathy be identified?
Reduced ankle reflex, positive sciatic nerve stretch test, sensory loss of posterior leg and lateral foot
734
What is first line tx for lower back pain?
NSAIDS (with PPI)
735
What is first line Ix for non specific back pain?
MRI (can see neurological and soft tissue structures)
736
What spinal nerves form the sciatic nerve?
L4-S3
737
What nerves does the sciatic nerve divide into?
Tibial nerve and common peroneal nerve
738
What are the symptoms of sciatica?
Unilateral pain from the buttock down the back of thigh to below the knee or feet. Paraesthesia, numbness, motor weakness
739
What cancers most commonly metastasise to bone?
Breast, Lung, Thyroid, Kidney, Prostate
740
What is the sciatic stretch test?
Diagnoses sciatica: patient lies on their back with leg straight up, then dorsiflexes ankle. This should cause sciatic nerve root irritation
741
What is cauda equina?
Compression of lumbosacral nerve roots beneath the spinal cord
742
What is the most common cause of cauda equina?
Central disc prolapse at L4/L5 or L5/S1
743
What are possible causes of cauda equina?
Central disc prolapse, tumours, infection, trauma, haematoma
744
What is the presentation of cauda equina?
Lower back pain, bilateral sciatica, reduced sensation (saddle distribution), decreased anal tone, urinary dysfunction
745
What is the investigation in cauda equina?
Urgent MRI
746
What is the treatment of cauda equina and possible complications?
Emergency decompression, or there may be permanent neurological dysfunction
747
What level does the spinal cord terminate?
L2/L3
748
What is a key feature of metastatic spinal cord compression?
Back pain that is worse on coughing or straining
749
What is the treatment of metastatic spinal cord compression?
High dose dexamethasone to reduce swelling, analgesia, surgery, chemo, radiotherapy
750
Why is neuropathic pain difficult to treat?
Responds poorly to standard analgesia
751
What are examples of neuropathic pain?
Diabetic neuropathy, post herpetic neuralgia, trigeminal neuralgia, prolapsed intervertebral disc
752
What is unique about treatment for neuropathic pain?
Monotherapy: if not working, drugs should be switched, not added
753
What are signs of neuropathic pain?
Burning, tingling, pins and needles, electric shocks, loss of sensation to touch of the affected area
754
What are potential side effects of NSAIDs?
Gastritis with dyspepsia, stomach ulcers, asthma exacerbation, HTN, renal impairment, coronary artery disease
755
What are potential side effects of opioids?
Constipation, skin itching, nausea, altered mental state, respiratory depression
756
What is the treatment of patients with chronic primary pain?
No analgesia should be started, only depression
757
What questionnaire assesses neuropathic pain?
DN4 questionnaire
758
What occurs in a dorsal column lesion?
Loss of vibration and proprioception
759
What occurs in a spinothalamic tract lesion?
Loss of pain, sensation and temperature
760
What are the symptoms of a central cord lesion?
Flaccid paralysis of the upper limbs
761
How does infarction of the spinal cord present?
Dorsal column signs (loss of proprioception and fine touch discrmination)
762
What is Froment's sign and what is it testing for?
Ulnar nerve palsy. Ask to 'break the O'
763
What nerves innervate the anal sphincter?
S2-4
764
What is the area of lesion in Huntington's Chorea?
Striatum
765
What is presentation of anterior cord syndrome?
Motor paralysis below the level of the lesion, loss of pain and temperature at and below the lesion
766
What causes anterior cord syndrome?
anterior spinal artery injury, usually due to flexion/compression injury
767
What is the pattern of brown sequard syndrome?
Ipsilateral LMN paralysis at level of the segment, ipsilateral loss of fine touch, proprioception and vibration. Contralateral loss of pain and temperature below level of lesion
768
Which spinal tract crosses over?
Spinothalamic: decussates at the level of the spinal cord
769
What is narcolepsy?
A condition where the brain loses its ability to regulate the sleep-wake cycle
770
What is the pathophysiology of narcolepsy?
Low levels of orexin, a protein which is responsible for controlling appetite and sleep patterns
771
What are the features of narcolepsy?
Excessive day time sleepiness, cataplexy, distrupted nighttime sleep, hypnagogic/hypnopompic hallucinations, sleep paralysis
772
What is cataplexy?
Conscious collapse caused by muscle atonia, often in response to sudden emotion. Seen in narcolepsy
773
What is the investigations for narcolepsy?
Multiple sleep latency EEG, CSF levels of orexin
774
What is the treatment of narcolepsy?
Daytime stimulants (modafinil) and sodium oxybate for improving nighttime sleep
775
What is menieres disease?
Long term inner ear disorder
776
What is the typical triad of Menieres disease?
Hearing loss, vertigo, tinnitus. Unilateral
777
What type of hearing loss is seen in Menieres?
Sensorineural, associated with vertigo attacks and low frequency
778
What is the pathophysiology of Menieres disease?
Excessive pressure and buildup of endolymph in the labryinth of the inner ear. Causes a higher pressure than normal, disrupts sensory signals. This is called endolymphatic hydrops
779
What are symptoms other than the usual triad in Menieres?
Sensation of fullness in the ear, unexplained falls ('drop attacks'), imbalance, nystagmus
780
What triggers vertigo in Menieres?
Nothing: random
781
How long do episodes last in menieres disease?
Minutes to hours
782
How can driving be affected in patients with Menieres?
They should inform the DVLA and cease driving until symptoms are controlled
783
What is the acute treatment of Menieres?
Prochlorperazine
784
What is the prevention of Menieres disease attacks?
Betahistine
785
What is the most common mononeuropathies?
Median nerve
786
What is myelopathy?
Pathology involving the spinal cord
787
What is syringomyelia?
Collection of CSF in the spinal cord
788
What is syringobulbia?
Fluid filled cavity within the medulla of the brainstem. Often an extension of syringomyelia
789
What are causes of syringomyelia?
Chiari malformation, trauma, tumours, idiopathic
790
What are the symptoms of syringomyelia?
'Cape like' distribution: loss of sensation to temperature, preservation of light touch, proprioception, vibration. Patients might burn their hands without realising. Neuropathic pain, spastic weakness
791
What is the investigations of syringomyelia?
MRI
792
What is the treatment of syringomyelia?
The cause of syrinx, or a shunt
793
What is Charcot-Marie-Tooth disease?
Most common hereditary peripheral neuropathy
794
What is the inheritance of Charcot Marie Tooth?
Autosomal dominant
795
What are the symptoms of Charcot-Marie-Tooth?
Mostly motor symptoms. High foot arches, distal muscle wasting (inverted champagne muscle legs), lower leg weakness (foot drop), weakness in hands, reduced reflexes, reduced muscle tone, peripheral sensory loss
796
How do you differentiate a 3rd nerve palsy that requires medical vs surgical treatment?
If there is a dilated pupil alongside 'down and out', it requires surgical management. This is controlled by parasympathetic fibres, which are on the surface of the nerve, which suggests a compression of the nerve (tumour or bleed).
