Neurology Flashcards
1
Q
How does sodium valproate work?
A
Increases GABA activity
2
Q
What are some adverse effects of sodium valproate?
A
- tremor
- P450 inhibitor
- hepatotoxicity
- alopecia
- weight gain
- nausea
- hyponatraemia
- pancreatitis
- teratogenicity
- hyperammonemic encephalopathy
- thrombocytopenia
3
Q
What are the teratogenic effects of sodium valproate?
A
- neural tube defects
- neurodevelopmental delay in children
Pregnancy prevention programme in place to avoid this.
4
Q
What are the typical features of post LP headache?
A
- usually develops within 24-48 hours following LP but may occur up to one week later
- may last several days
- worsens with upright position/improves with recumbent position
5
Q
What is the management for a post LP headache?
A
-supportive initially (analgesia, rest)
- if pain continues for more than 72 hours then specific treatment is indicated, to prevent subdural haematoma e.g blood patch, epidural saline and intravenous caffeine
6
Q
What are the features of vestibular neuronitis?
A
- recurrent vertigo attacks lasting hours or days
- nausea and vomiting
- horizontal nystagmus
- no hearing loss or tinnitus
7
Q
What is the management for vestibular neuronitis?
A
- buccal or intramuscular prochlorperazine is often used to provide rapid relief for severe cases.
- a short oral course of prochlorperazine, or an antihistamine (cinnarizine, cyclizine, or promethazine) may be used to alleviate less severe cases
- vestibular rehabilitation exercises are the preferred treatment for patients who experience chronic symptoms
8
Q
What are the features of a cluster headache?
A
- intense sharp, stabbing pain around one eye
- pain typically occurs once or twice a day, each episode lasting 15 mins - 2 hours
- restless and agitated due to the severity
- clusters typically last 4-12 weeks
- accompanied by redness, lacrimation, lid swelling of eye
- nasal stuffiness
- miosis and ptosis in a minority
9
Q
What is the investigation of choice for cluster headache?
A
MRI with gadolinium contrast
10
Q
What is the acute management for cluster headache?
A
- 100% oxygen (80% response rate within 15 minutes)
- subcutaneous triptan (75% response rate within 15 minutes)
11
Q
What is the prophylaxis for cluster headache?
A
- verapamil
there is also some evidence to support a tapering dose of prednisolone
12
Q
Which area of the hypothalamus is affected in someone with a craniopharyngioma?
A
ventromedial area of the hypothalamus
(controls satiety)
13
Q
What is the management for an acute relapse of MS?
A
High-dose steroids (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten the length of an acute relapse.
14
Q
What are the first line treatments for spasticity in MS?
A
Baclofen and Gabapentin
15
Q
What drugs are used to reduce the risk of relapse in MS?
A
- natalizumab
- ocrelizumab
-fingolimod - beta-interferon
- glatiramer acetate
16
Q
What is the first line treatment for fatigue in MS?
A
trial of amantadine
17
Q
What is the first line treatment for oscillopsia in MS?
A
Gabapentin
18
Q
Which medications exacerbate myasthenia gravis?
A
- penicillamine
- quinidine, procainamide
- beta-blockers
- lithium
- phenytoin
- antibiotics: gentamicin, macrolides, quinolones, tetracyclines
19
Q
What are the typical features of multiple system atrophy?
A
- parkinsonism
- autonomic disturbance
erectile dysfunction: often an early feature
postural hypotension
atonic bladder - cerebellar signs
20
Q
What are the features of Otosclerosis?
A
- conductive deafness
- tinnitus
- tympanic membrane
the majority of patients will have a normal tympanic membrane
10% of patients may have a ‘flamingo tinge’, caused by hyperaemia - positive family history
21
Q
What is the management of otosclerosis?
A
- Hearing aid
- Stapedectomy
22
Q
What are examples of drugs that cause peripheral neuropathy?
A
amiodarone
isoniazid
vincristine
nitrofurantoin
metronidazole
23
Q
What are the classical features of vestibular schwannoma?
A
- Vertigo
- hearing loss (unilateral)
- tinnitus
- Absent corneal reflex
24
Q
In which condition are bilateral vestibular schwannomas seen?
A
Neurofibromatosis 2
25
what is the first line for prevention of Meniere's attacks?
Betahistine
26
What are the typical features of Meniere's disease?
- recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom
- a sensation of aural fullness or pressure is now recognised as being common
- nystagmus and a positive Romberg test
- episodes last minutes to hours
- unilateral
27
what medication is used for the treatment of acute attacks of Meniere's disease?
buccal or intramuscular prochlorperazine
28
What are some visual symptoms of MS?
- optic neuritis
- optic atrophy
- Uhthoff's phenomenon: worsening of vision following rise in body temperature
- internuclear ophthalmoplegia
29
What are some sensory symptoms of MS?
- pins and needles
- numbness
- trigeminal neuralgia
- Lhermitte's syndrome: paraesthesiae in limbs on neck flexion
30
What are some motor symptoms of MS?
spastic weakness: most commonly seen in the legs
31
What are some cerebellar symptoms of MS?
- ataxia: more often seen during an acute relapse than as a presenting symptom
- tremor
32
What are some other symptoms of MS?
- urinary incontinence
- sexual dysfunction
- intellectual deterioration
33
Which medication can lead to the reactivation of John Cunningham (JC) viru, leading to progressive multifocal leukoencephalopathy (PML)?
- Natalizumab
34
What are the cerebellar features of Lateral medullary syndrome?
- Ataxia
- Nystagmus
35
What are the brainstem features of Lateral medullary syndrome?
- Ipsilateral: dysphagia, facial numbness, cranial nerve palsy e.g. Horner's
- Contralateral: limb sensory loss
36
What are the features of conduction aphasia?
- Fluent speech
- Poor repetition
- Aware of the errors
- Comprehension is normal
37
What are the features of Broca's aphasia?
- Non fluent speech
- Impaired repetition
- Comprehension is normal
- lesion usually within L superior MCA
38
What are the features of Wernicke's aphasia?
- Fluent speech
- Speech does not make sense - word salad
- Impaired comprehension
39
Which dopamine receptor agonist used in PD is least associated with pulmonary, retroperitoneal and pericardial fibrosis?
Ropinirole
40
Which organism classically triggers Guillian barre syndrome?
Campylobacter jejuni
41
What is the first line treatment for Guillain-barre syndrome
IV Immunoglobulins
42
Which antibodies are associated with Lambert-Eaton syndrome?
Anti-VGCC (voltage gated calcium channels)
43
What are some examples of drugs associated with idiopathic intracranial hypertension?
- combined oral contraceptive pill
- steroids
- tetracyclines
- retinoids (isotretinoin, tretinoin) / vitamin A
- lithium
44
What are the features of Idiopathic Intracranial Hypertension?
headache
blurred vision
papilloedema (usually present)
enlarged blind spot
sixth nerve palsy may be present
45
What is the management of Idiopathic Intracranial Hypertension?
- weight loss
- semaglitide and topiramate may be considered by specialists.
- carbonic anhydrase inhibitors e.g. acetazolamide
- repeated lumbar puncture may be used as a temporary measure but is not suitable for longer-term management
- surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve.
- lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
46
What syndrome are anti-GAD antibodies associated with?
Stiff person syndrome
47
What condition are anti-Yo antibodies associated with?
Cerebellar syndrome
Ovarian and breast Cancer
48
What condition are anti-Hu antibodies associated with?
Small cell lung carcinoma
Neuroblastomas
Sensory neuropathy
Cerebellar syndrome
Encephalomyelitis
49
What condition are anti-Ri antibodies associated with?
Breast cancer
Small cell lung cancer
Ocular opsoclonus-myoclonus
50
What condition is associated with Purkinje cell antibodies?
Peripheral neuropathy in breast cancer
51
What are the ket features of essential tremor?
- Worse when arms outstretched
- Improves with rest and alcohol
- most common cause of head tremor
52
What is the first line treatment for essential tremor?
Propranolol
53
What are the features of cervical spondylitis myopathy?
- mix of motor weakness, sensory loss and bladder/bowel dysfunction
- neck pain
- wide-based, ataxic or spastic gait
- UMN weakness in the lower legs - increased reflexes, increased tone and upgoing plantars
- bladder dysfunction e.g. urgency, retention
54
When can you consider stopping anti-epileptic drugs?
- Seizure free for > 2years
- AEDs stopped over 2-3months
55
What are some symptoms of degenerative cervical myopathy?
- Pain (affecting the neck, upper or lower limbs)
- Loss of motor function (loss of digital dexterity) - weakness/stiffness leading to impaired gait and imbalance
- Loss of sensory function
- Loss of autonomic function (urinary or faecal incontinence and/or impotence)
- Hoffman's sign: is a reflex test to assess for cervical myelopathy. It is performed by gently flicking one finger on a patient's hand. A positive test results in reflex twitching of the other fingers on the same hand in response to the flick.
56
Which condition is associated with Anti-NMDA receptor encephalitis?
Ovarian teratoma
57
What are the cutaneous features of tuberous sclerosis?
- depigmented 'ash-leaf' spots which fluoresce under UV light
- roughened patches of skin over lumbar spine (Shagreen patches)
- adenoma sebaceum (angiofibromas): butterfly distribution over nose
- fibromata beneath nails (subungual fibromata)
- cafe-au-lait spots* may be seen
58
What are the neurological features of tuberous sclerosis?
- Epilepsy
- Developmental delay
- Intellectual impairment
59
What are the features of neuroleptic malignant syndrome?
- pyrexia
- muscle rigidity
- autonomic lability: typical features include hypertension, tachycardia and tachypnoea
- agitated delirium with confusion
60
What is the management of neuroleptic malignant syndrome?
- Stop antipsychotic
- IVF to prevent renal failure
- Bromocryptine
- Dantrolene may be used in selected cases
61
What is the inheritance and genetic abnormality in Von Hippel-Lindau syndrome?
- Autosomal dominant
- Abnormality in the VHL gene located on short arm of chromosome 3
62
What are the features of Von Hippel-Lindau syndrome?
- cerebellar haemangiomas: can cause SAH
- retinal haemangiomas: vitreous haemorrhage
- renal cysts (premalignant)
- phaeochromocytoma
- extra-renal cysts: epididymal, pancreatic, hepatic
endolymphatic sac tumours
- clear-cell renal cell carcinoma
63
What is internuclear ophthalmoplegia?
- a cause of horizontal disconjugate eye movement
- lesion in the medial longitudinal fasciculus (MLF)
controls horizontal eye movements by interconnecting the IIIrd, IVth and VIth cranial nuclei
located in the paramedian area of the midbrain and pons
64
What are some causes of internuclear ophthalmoplegia?
- MS
- Vascular disease
64
What are the features of internuclear ophthalmoplegia?
- Impaired adduction of the eye on the same side as the lesion
- horizontal nystagmus of the abducting eye on the contralateral side
65
What is syringomyelia?
Collection of CSF within spinal cord resulting in compression and damage to nerve fibres.
66
What are the ket features of Syringomyelia?
- a 'cape-like' (neck, shoulders and arms)
loss of sensation to temperature but the preservation of light touch, proprioception and vibration
- spastic weakness (predominantly of the lower limbs)
- neuropathic pain
- upgoing plantars
- autonomic features
- Horner's syndrome due to compression of the sympathetic chain, but this is rare, bowel and bladder dysfunction
- scoliosis will occur over a matter of years if the syrinx is not treated
67
What are the key features of motor neurone disease?
- Asymmetrical limb weakness
- Mixture of UMN and LMN signs
- Fasciculations
- wasting of the small hand muscles/tibialis anterior
- absence of sensory signs/symptoms
- doesn't affect ocular muscles
- no cerebellar signs
- abdominal reflexes usually preserved
68
What is cataplexy?
Sudden and transient episode of muscle weakness following laughing/anger/suprise.
Associated with narcolepsy
69
Where is the chemoreceptor trigger zone?
Medulla oblongata
70
What are the characteristic features of Guillain-Barré syndrome?
- the weakness is classically ascending i.e. the legs are affected first
- reflexes are reduced or absent
- sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs
71
What are some other features of Guillain- Barre Syndrome?
- history of gastroenteritis
- respiratory muscle weakness
- cranial nerve involvement
diplopia
bilateral facial nerve palsy
oropharyngeal weakness is common
- autonomic involvement
urinary retention
diarrhoea
72
What are some causes of demyelinating peripheral neuropathy?
- Guillain-Barre syndrome
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- amiodarone
- hereditary sensorimotor neuropathies (HSMN) type I
- paraprotein neuropathy
73
What are some causes of axonal peripheral neuropathy?
- alcohol
- diabetes mellitus
- vasculitis
- vitamin B12 deficiency
- hereditary sensorimotor neuropathies (HSMN) type II
74
What is the treatment for a brain abscess?
IV 3rd generation cephalosporin and metronidazole
75
How does a brain abscess appear on CT scan?
intracerebral lesion with an outer hypodense and inner hyperdense rim
76
What is the treatment for Ramsay Hunt Syndrome/
Oral aciclovir and oral corticosteroids
77
What medication is contraindicated in absence seizures?
Carbamazepine
78
What is the mechanism of action of phenytoin?
Binds to sodium channels increasing their refractory period.
79
What are some acute side effects of phenytoin?
- Initially: dizziness, diplopia, nystagmus, slurred speech, ataxia
- later: confusion, seizures
80
What are some chronic side effects of phenytoin?
- common: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness
- megaloblastic anaemia (secondary to altered folate metabolism)
- peripheral neuropathy
- enhanced vitamin D metabolism causing osteomalacia
- lymphadenopathy
- dyskinesia
81
How is BPPV diagnosed?
Dix-Hallpike manoeuvre
82
How is BPPV treated?
Epley manoeuvre
83
What medication can be used to aid prognosis in MND?
Riluzole
84
What are some typical features of a parietal lobe lesion?
- sensory inattention
- apraxias
- astereognosis (tactile agnosia)
- inferior homonymous quadrantanopia
- Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
85
What are some features of an occipital lobe lesion?
- homonymous hemianopia (with macula sparing)
- cortical blindness
- visual agnosia
86
What are some features of a temporal lobe lesion?
- Wernicke's aphasia: this area 'forms' the speech before 'sending it' to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent
- superior homonymous quadrantanopia
- auditory agnosia
- prosopagnosia (difficulty recognising faces
87
What are some features of frontal lobe lesions?
- expressive (Broca's) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting
- disinhibition
- perseveration
- anosmia
- inability to generate a list
88
What are some features of cerebellar lesions?
- midline lesions: gait and truncal ataxia
- hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus
89
What intervention has the best prognostic benefit in MND?
Non Invasive ventilation
90
What are the general features of myotonic dystrophy?
- myotonic facies (long, 'haggard' appearance)
- frontal balding
- bilateral ptosis
- cataracts
- dysarthria
91
What are the key features of DM1 myotonic dystrophy?
- DMPK gene on chromosome 19
- Distal weakness more prominent
92
What are the key features of DM2 myotonic dystrophy?
- ZNF9 gene on chromosome 3
- Proximal weakness more prominent
- Severe congenital form not seen
93
What is Miller-Fisher syndrome?
- Variant of Gillian-Barre Syndrome
- Features include: ophthalmoplegia, areflexia and ataxia.
- anti-GQ1b antibodies are present in 90% of cases
- descending paralysis usually
94
What is the first line treatment for trigemini neuralgia?
Carbamazepine
95
What are the key features of Trimgeminal Neuralgia?
- unilateral disorder
- brief electric shock-like pains
- abrupt in onset and termination
- limited to one or more divisions of the trigeminal nerve
- commonly evoked by light touch, including washing, shaving, smoking, talking, and brushing the teeth (trigger factors).
- small areas in the nasolabial fold or chin may be particularly susceptible to the precipitation of pain (trigger areas)
- the pains usually remit for variable periods
96
What are some red flag symptoms in trigeminal neuralgia which suggest a serious underlying cause?
- Sensory changes
- Deafness or other ear problems
- History of skin or oral lesions that could spread perineurally
- Pain only in the ophthalmic division of the trigeminal nerve (eye socket, forehead, and nose), or bilaterally
- Optic neuritis
- A family history of multiple sclerosis
- Age of onset before 40 years
97
What receptors do apomorphine work on?
Dopamine receptors
98
What is the most common complication of meningitis?
Sensorineural hearing loss
99
What are some common adverse effects of levodopa?
dyskinesia
'on-off' effect
postural hypotension
cardiac arrhythmias
nausea & vomiting
psychosis
reddish discolouration of urine upon standing
100
How long should someone stop driving for post stroke/TIA?
1month
101
What conditions can cause miosis (small pupils)?
Horner's syndrome
Argyll-Robertson pupil
senile miosis
pontine haemorrhage
congenital
102
What drugs can cause miosis?
opiates
parasympathomimetics: pilocarpine
organophosphate toxicity
103
What is a rare side effect of lamotrigine?
Steven-Johnson syndrome
104
What are some causes of bilateral facial nerve palsy?
- sarcoidosis
- Guillain-Barre syndrome
- Lyme disease
- bilateral acoustic neuromas (as in neurofibromatosis type 2)
- Bell's palsy (relatively common it accounts for up to 25% of cases of bilateral palsy, but this represents only 1% of total Bell's palsy cases)
105
What are some causes of unilateral facial nerve palsy?
Bell's palsy
Ramsay-Hunt syndrome (due to herpes zoster)
acoustic neuroma
parotid tumours
HIV
multiple sclerosis
diabetes mellitus
106
What are the key features of an extradural haemorrhage?
- Lucid interval
- Raised intracranial pressure
- Acceleration-deceleration trauma or blow to side of head
107
What are the features of Facioscapulohumeral muscular dystrophy (FSHMD)?
- facial muscles are involved first - difficulty closing eyes, smiling, blowing etc
- weakness of the shoulder and upper arm muscles
- abnormal prominence of the borders of the shoulder blades - 'winging'
- lower limb: hip girdle weakness, foot drop
108
What virus characteristically affects the temporal lobes?
Herpes Simplex Virus
109
What receptors does Baclofen work on?
GABA
110
What are some features of a temporal lobe seizure?
Lip smacking
Grabbing
Plucking
Post-ictal dysphasia
111
What is the inheritance pattern of essential tremor?
Autosomal Dominant
112
What condition is associated with a painful third nerve palsy?
posterior communicating artery aneurysm
113
What are the ket features of a third nerve palsy?
Eye is deviated Down and out
Ptosis
Pupil may be dilated
114
What are some causes of a third nerve palsy?
- Diabete mellitus
- vasculitis e.g. temporal arteritis, SLE
- false localizing sign* due to uncal herniation through tentorium if raised ICP
- posterior communicating artery aneurysm
- cavernous sinus thrombosis
- Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia
- other possible causes: amyloid, multiple sclerosis
115
What are some absolute contraindications to thrombolysis?
- Previous intracranial haemorrhage
- Seizure at onset of stroke
- Intracranial neoplasm
- Suspected subarachnoid haemorrhage
- Stroke or traumatic brain injury in preceding 3 months
- Lumbar puncture in preceding 7 days
- Gastrointestinal haemorrhage in preceding 3 weeks
- Active bleeding
- Oesophageal varices
- Uncontrolled hypertension >200/120mmHg
116
What are some relative contraindications to thrombolysis?
- Pregnancy
- Concurrent anticoagulation (INR >1.7)
- Haemorrhagic diathesis
- Active diabetic haemorrhagic retinopathy
- Suspected intracardiac thrombus
- Major surgery / trauma in the preceding 2 weeks
117
What are some complications of meningitis?
-Sensorineural hearing loss
- seizures
- focal neurological deficit
- infective (sepsis/intracerebral abscess)
- pressure (brain herniation/hydrocephalus)
118
What are the features of Progressive Supranuclear Palsy?
- postural instability and falls
- patients tend to have a stiff, broad-based gait
-impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs)
- parkinsonism
bradykinesia is prominent
- cognitive impairment
primarily frontal lobe dysfunction
119
Which part of the brain does hemiballismus affect?
Subthalamic nucleus of the basal ganglia
120
Which drug is used to treat tremor in drug induced Parkinsonism?
Benzhexol (trihexyphenidyl)
121
Which areas of the spinal cord are affected in subacute combined degeneration of the spinal cord?
dorsal columns, lateral corticospinal tracts and spinocerebellar tracts..
122
What are the features of Wernicke's encephalopathy?
Confusion
Ataxia
Nystagmus
Ophthamoplegia
PEripheral
Neuropathy
123
What are key features of hemiballism?
involuntary, sudden, jerking movements which occur contralateral to the side of the lesion
124
What is the main treatment for hemiballism movements?
Antidopaminergic agents e.g Haloperidol
125
what is the typical presentation of variant CJD?
-younger patient with progressive dementia (less rapid the sporadic CJD)
- myoclonus
- later stages, mutism and vertical upgaze palsy (found in 50%).
- MRI brain reveals a characteristic 'hockey stick sign'
126
What is the most common psychiatric presentation in Parkinson's?
Depression
127
What are the key features of brachial neuritis?
acute onset of unilateral severe pain followed by shoulder and scapular weakness several days later
128
What causes of peripheral neuropathy are predominantly sensory?
diabetes
uraemia
leprosy
alcoholism
vitamin B12 deficiency
amyloidosis
129
What causes of peripheral neuropathy are predominantly motor?
Guillain-Barre syndrome
porphyria
lead poisoning
hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
chronic inflammatory demyelinating polyneuropathy (CIDP)
diphtheria
130
What suggest idiopathic Parkinsons rather than drug induced?
Asymmetrical symptoms
131
What is the Barthel index?
scale that measures disability or dependence in activities of daily living in stroke patients
132
What drugs can commonly cause gingival hyperplasia?
Phenytoin
CCBs
Ciclosporin
133
A lesion in which area of the brain can cause astereognosis?
Parietal lobe
134
What are some causes of chorea?
Huntington's disease
Wilson's disease
ataxic telangiectasia
SLE
anti-phospholipid syndrome
rheumatic fever: Sydenham's chorea
drugs: oral contraceptive pill, L-dopa, antipsychotics
neuroacanthocytosis
pregnancy: chorea gravidarum
thyrotoxicosis
polycythaemia rubra vera
carbon monoxide poisoning
cerebrovascular disease
135
What are the key features of Klumpke's palsy?
- classically occurs as a birth injury, or when falling from a tree and grabbing onto a branch whilst falling
- Intrinsic hand muscles and flexors of wrist and fingers affected
- claw hand
- sensory loss in C8/T1 dermatomes
136
What is a common consequence of subarachnoid haemorrhage?
Syndrome of inappropriate anti-diuretic hormone (SIADH).
137
What is autoinduction?
process through which a drug induces the enzymes responsible for its own metabolism, thereby reducing its efficacy.
138
What drug is an autoinducer?
Carbamazepine
139
What are some causes of Parkinsonism?
Parkinson's disease
drug-induced e.g. antipsychotics, metoclopramide*
progressive supranuclear palsy
multiple system atrophy
Wilson's disease
post-encephalitis
dementia pugilistica (secondary to chronic head trauma e.g. boxing)
toxins: carbon monoxide, MPTP
140
What is the treatment for myasthenia crisis?
IV immunoglobulins (1st line)
Plasmapheresis (expensive but quicker)
141
Where is the lesion if a person is unable to read but able to write and understand normally?
Corpus callosum
142
What virus can natalizumab cause reactivation of?
JC virus
143
What is the preferred form of nutritional support in MND
PEG
144
What are the key characteristics of sporadic CJD?
Progressive onset dementia and myoclonus
145
What is the 1st, 2nd and 3rd line treatment for focal seizures?
1st - Lamotrigine or levetiracetam
2nd - Levetiracetam or lamotrigine
3rd - Carbamazepine
146
Features of a good prognosis in MS?
female sex
age: young age of onset (i.e. 20s or 30s)
relapsing-remitting disease
sensory symptoms only
long interval between first two relapses
complete recovery between relapses
147
Which MRI sequence is used in diagnosis of MS?
MRI FLAIR
148
What cancer is associated with Von-Hippel-Lindau syndrome?
Renal cell carcinoma
149
Where is the gene defect located that causes Von-Hippel-Lindau Syndrome?
Short arm of chromosome 3
150
What drug can precipitate acute angle closure glaucoma?
Topiramate
151
What MRI findings are expected in a patient with Wernicke's encephalopathy?
enhancement of the mamillary bodies due to petechial haemorrhages
152
What are the features of transient global amnesia?
- acute onset of anterograde amnesia (the inability to form new memories). - It usually resolves within 24 hours.
- may appear anxious an repeatedly ask the same question
153
What is the appropriate management for someone with degenerative cervical myelopathy?
Referral for decompressive surgery
154
What are the common features of intracranial venous thrombosis?
headache (may be sudden onset)
nausea & vomiting
reduced consciousness
155
What are the investigations for intracrhail venous thrombosis?
- MRI venography is the gold standard
CT venography is an alternative
- non-contrast CT head is normal in around 70% of patients
- D-dimer levels may be elevated
156
What condition is associated with bilateral vestibular schwannomas?
Neurofibromatosis 2
157
What commonly causes cerebellar abscesses?
Mastoiditis
Sinusitis infections
158
Whta is one o e early complications of SAH?
Re-bleeding.
If suspected then re scan (Ct head)
159
What conditions are associated with berry aneurysms?
Adult polycystic kidney disease
Coarctation of the aorta
Ehlers-Danlos syndrome
Hypertension
160
What is often the earliest and most frequent manifestation of Von Hippel Lindau syndrome?
Retinal haemangiomas presenting with vitreous haemorrhage
161
What are the neurological features of Friedrich's ataxia?
absent ankle jerks/extensor plantars
cerebellar ataxia
optic atrophy
spinocerebellar tract degeneration
162
What is the inheritance pattern and gene abnormality in Friedrich's ataxia?
- Autosomal recessive
- Trinucleotide repeat disorder
- GAA repeat in the X25 gene on chromosome 9 (frataxin).
163
What are the features of a 4th nerve palsy?
-vertical diplopia
(classically noticed when reading a book or going downstairs)
- subjective tilting of objects (torsional diplopia)
- the patient may develop a head tilt, which they may or may not be aware of
- when looking straight ahead, the affected eye appears to deviate upwards and is rotated outwards
164
What are the key features of acute angle closure glaucoma?
- Painful red eye
- blurred vision
- nausea, and vomiting
- 'haloes' in vision.
165
What is the mechanism of action of triptans?
5-HT1 receptor agonist
166
What are the features of Charcot-Marie-Tooth Disease?
There may be a history of frequently sprained ankles
Foot drop
High-arched feet (pes cavus)
Hammer toes
Distal muscle weakness
Distal muscle atrophy
Hyporeflexia
Stork leg deformity
167
What is the mechanism of action for procyclidine?
Anti-muscarinic
168
What are the key features of idiopathic intracranial hypertension?
headache
blurred vision
papilloedema (usually present)
enlarged blind spot
sixth nerve palsy may be present
169
What are the risk factors for idiopathic intracranial hypertension?
obesity
female sex
pregnancy
drugs
combined oral contraceptive pill
steroids
tetracyclines
retinoids (isotretinoin, tretinoin) / vitamin A
lithium
170
What is the management of idiopathic intracranial hypertension?
Weight loss
- carbonic anhydrase inhibitors e.g. acetazolamide
- topiramate is 2nd line
- repeated lumbar puncture may be used as a temporary measure
- surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve.
- A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure
171
What is used to measure respiratory function in Guillain Barre Syndrome?
Forced vital capacity
172
What is CADASIL?
Cerebral Autosomal Dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy
-mutation in the NOCTH3 gene.
- onset of migraines in middle age, followed by recurrent TIAs and strokes, and resulting in neuro-cognitive decline, psychiatric problems and dementia.
- MRI often shows multiple widespread hyper-intense lesions in the white matter, basal ganglia, thalamus and pons.
173
What are the associated affects of an anterior cerebral artery stroke?
Contralateral hemiparesis and sensory loss, lower extremity > upper
174
What are the associated affects of a middle cerebral artery stroke?
- Contralateral hemiparesis and sensory loss, upper extremity > lower
- Contralateral homonymous hemianopia
- Aphasia
175
What are the affects of a posterior cerebral artery stroke?
- Contralateral homonymous hemianopia with macular sparing
- Visual agnosia
176
What are the affects of a basilar artery stroke?
'Locked-in' syndrome
177
What are the affects of Weber's syndrome (branches of the posterior cerebral artery that supply the midbrain)?
- Ipsilateral CN III palsy
- Contralateral weakness of upper and lower extremity
178
What are the affects of Posterior inferior cerebellar artery stroke (lateral medullary syndrome, Wallenberg syndrome)?
- Ipsilateral: facial pain and temperature loss
- Contralateral: limb/torso pain and temperature loss
- Ataxia
- Nystagmus
179
What are the affects of an anterior inferior cerebellar artery stroke (lateral pontine syndrome)?
- Ipsilateral: facial paralysis and deafness
- Contralateral: limb/torso pain and temperature loss
- Ataxia
-Nystagmus
180
What are the affects of a retinal artery stroke?
Amaurosis fugax
181
What are the radiological findings of transverse myelitis?
Hyperintense T2 signal extending across the spinal cord
182
What are the features of Bell's palsy?
- LMN facial nerve palsy → forehead affected
- post-auricular pain (may precede paralysis)
- altered taste
- dry eyes
- hyperacusis (sensitivity to sounds)
183
What is the management of Bells' Palsy?
Prednisolone within 72hrs
Acyclovir in severe cases alongside steroids
Eye care
184
What are the features and onset of neuroleptic malignant syndrome?
- pyrexia
- muscle rigidity
- autonomic lability: typical features include hypertension, tachycardia and tachypnoea
- agitated delirium with confusion
-hours to days of starting an antipsychotic
185
What is the treatment for otitis external?
Topical corticosteroid and aminoglycoside
186
Why may paracetamol at regular dose may not be effective at relieving migraine?
Migraine causes delayed gastric emptying.
Often combined with prokinetics e.g. metoclopramide
187
What is the mechanism of action of pyridostigmine?
Long acting anti cholinesterase inhibitor
188
What are some side effects associated with dopamine receptor agonists e.g cabergoline?
Pulmonary, cardiac and retroperitoneal fibrosis
Daytime somnolence
Nasal congestion
Postural hypotension
Impulse control disorders
189
What are the key features of Miller Fischer Syndrome?
Areflexia
Ataxia
Ophthalmoplegia
190
What are the features and inheritance pattern of Charcot-Marie-Tooth Disease?
- Autosomal dominant
- due to defect in PMP-22 gene (which codes for myelin)
- features often start at puberty
- motor symptoms predominate
- distal muscle wasting, pes cavus, clawed toes
- foot drop, leg weakness often first features
191
What medication is used to prevent vasospasm in aneurysmal subarachnoid haemorrhages?
Nimodipine
192
What antibodies are strongly associated with Miller-Fischer syndrome?
Anti-GQ1b antibodies (present in 90% of cases)
193
Which anti-epileptic medication is most likely to cause visual field defects?
Vigabatrin
194
How much folic acid should a pregnant person who has epilepsy take?
5mg folic acid once daily
195
What can be used to aid drooling in Parkinsons?
glycopyrronium bromide
196
What is Jacksonian march with secondary generalisation?
Type of focal seizure
Characteristically starts by affecting a peripheral body part such as a toe, finger or section of the lip and then spreads quickly 'marches' over the respective foot, hand or face
197
How do you differentiate between neuroleptic malignant syndrome and serotonin syndrome?
Myoclonus is only present in serotonin syndrome
198
What nucleotide repeats are present in Huntington's disease?
CAG
199
What nucleotide repeats are present in Friedrich's ataxia?
GAA
200
What nucleotide repeats are present in myotonic dystrophy?
CTG
201
What nucleotide repeats are present in fragile X syndrome?
CGG
202
What are the typical features of Huntington's?
chorea
personality changes (e.g. irritability, apathy, depression) and intellectual impairment
dystonia
saccadic eye movements
203
How do you distinguish between neuromyelitis optica and MS?
MS is not NMO-IgG seropositive.
204
What are the features of neuromyelitis optica?
Optic neuritis
Acute myelitis
Brain MRI not meeting criteria for MS at disease onset
Spinal cord MRI with contiguous T2-weighted signal abnormality extending over three or more vertebral segments
NMO-IgG seropositive status (The NMO-IgG test checks the existence of antibodies against the aquaporin 4 antigen.)
205
What can happen if you replace folate before B12 in a deficient patient?
Precipitate subacute degeneration of the spinal cord
206
What test is used to diagnose neurofibromatosis type 1?`
24hr urinary catecholamines
207
What neuropeptides are associated with narcolepsy?
low orexin (hypocretin) levels
208
