Neurology Flashcards
1
Q
Which type of motor neuron disease carries the worst prognosis?
A
Progressive bulbar palsy
2
Q
where is the median longitudinal fasciculus?
A
paramedian area of the midbrain and pons
3
Q
MOA of baclofen?
A
- GABA receptor agonist
- used to treat muscle spasticity in conditions such as multiple sclerosis, cerebral palsy and spinal cord injuries.
4
Q
features of Von Hippel Lindau?
A
auto dom condition -> neoplasia
- cerebellar haemangiomas -> subarachnoid haemorrhage
- retinal haemangiomas
- renal cysts
- phaeochromocytoma
- clear cell renal cell carcinoma
- extra renal cysts (hepatic, pancreatic, epididymal)
5
Q
hearing loss: vestibular neuronitis/ viral labyrinthitis?
A
vestibular neuronitis: NO hearing loss or tinnitus
6
Q
features of syringomyelia?
A
‘cape-like’ (neck and arms) loss of sensation to temperature, with preservation of light touch, proprioception and vibration
7
Q
Ix of syringomyelia?
A
full spine MRI with contrast to exclude a tumour or tethered cord.
+ brain MRI to exclude a Chiari malformation.
8
Q
DVLA time off driving after first unprovoked seizure?
A
- 6 months if brain imaging and EEG normal
- 12 months if any abnormality in ix
9
Q
DVLA time off driving after stroke/ TIA?
A
1 month off driving, may not need to inform DVLA if no residual neurological deficit
10
Q
DVLA time off driving after multiple TIAs?
A
3 months off driving and inform DVLA
11
Q
management of respiratory distress in MND?
A
NIV e.g. BIPAP
12
Q
current evidence based management of Bell’s palsy?
A
oral prednisolone
13
Q
which management options in MND prolongs survival?
A
- NIV: 7 months
- Riluzole: 3 months
14
Q
poor prognostic features of Guillain-Barre?
A
- age > 40 years
- poor upper extremity muscle strength
- previous history of a diarrhoeal illness (specifically Campylobacter jejuni)
- high anti-GM1 antibody titre
- need for ventilatory support
15
Q
topiramate and glaucoma?
A
topiramate can precipitate acute closed angle glaucoma
16
Q
Internuclear ophthalmoplegia: ie. Left eye unable to ADDUCT, where is the lesion?
A
LEFT medial longitudinal fasciculus
17
Q
first line of medical management for drooling of saliva in people with Parkinson’s disease
A
glycopyrronium bromide
*If not effective/ tolerated or contraindicated, consider botulinum toxin A.
18
Q
management of myoclonic seizures?
A
sodium valproate
2nd line: clonazepam, lamotrigine
19
Q
what anti epileptic might exacerbate myoclonic seizures?
A
carbamazepine
20
Q
Management of focal seizures?
A
carbamazepine or lamotrigine
21
Q
what antiepilpetic might exacerbate absence seizures?
A
carbamazepine
22
Q
management of absence seizures?
A
sodium valproate or ethosuximide
23
Q
management of generalised tonic clonic seizures
A
sodium valproate
2nd line: lamotrigine, carbamazepine
24
Q
features of anterior spinal artery occlusion?
A
- Bilateral spastic paresis
2. Bilateral loss of pain and temperature sensation
25
what tracts are affected in anterior spinal artery occlusion?
1. Lateral corticospinal tracts
| 2. Lateral spinothalamic tracts
26
what anti-emetics might cause QTc prolongation?
ondansetron
27
1st line mx of brain abscess?
- IV 3rd-generation cephalosporin (Ceftriaxone) + metronidazole
- dexamethasone to reduce pressure
- surgery to drain abscess
28
renal angiomyolipoma assoc w?
tuberous sclerosis
29
features of Miller Fischer syndrome?
- ophthalmoplegia, areflexia and ataxia.
| The eye muscles are typically affected first, "descending paralysis"
30
What antibodies are assoc w Miller Fischer syndrome?
anti-GQ1b antibodies
31
1st line management of benign essential tremor?
propranolol
32
when can you consider stopping antiepileptic drugs in epilepsy ?
if seizure free for >2 years. stop drugs over 2-3 months
33
1st line ix in suspected stroke?
non contrast CT head
34
features of parietal lobe brain lesion?
- sensory inattention
- apraxias (following commands)
- astereognosis (tactile agnosia)
- inferior homonymous quadrantanopia
- Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
35
features of occipital lobe lesion?
- homonymous hemianopia (with macula sparing)
- cortical blindness
- visual agnosia (not recognising objects)
36
features of temporal lobe lesion?
- Wernicke's aphasia
- superior homonymous quadrantanopia
- auditory agnosia
- prosopagnosia (difficulty recognising faces)
37
features of frontal lobe lesion?
- expressive (Broca's) aphasia
- disinhibition
- perseveration
- anosmia
- inability to generate a list
38
features of cerebellar lesion?
- midline lesions: gait and truncal ataxia
| - hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus
39
what area of the brain is implicated in Wernickes/ Korsakoffs?
Medial thalamus and mammillary bodies of the hypothalamus
40
What area of the brain is implicated in Huntington's chorea?
Striatum (caudate nucleus) of the basal ganglia
41
features of Kluver-Bucy syndrome
hypersexuality, hyperorality, hyperphagia, visual agnosia
- amygdala affected
42
what drugs might cause idiopathic intracranial HTN?
- COCP
- steroids
- tetracyclines
- vitamin A/ retinoids
- lithium
43
what medication options might be beneficial in idiopathic intracranial HTN?
- diuretics e.g. acetazolamide, topiramate
| - topiramate
44
what management for idiopathic intracranial HTN?
- weight loss
- repeated LPs
- Diuretics e.g. acetazolamide, topiramate
- surgery (ie. VP shunt, endovascular shunt in patients with transverse sinus stenoses)
45
what leukaemia is most associated with gingival hyperplasia?
AML
46
management of myasthenia crisis?
plasmapheresis + intravenous immunoglobulins
47
1st line management of myasthenia gravis?
pyridostigmine (acetylcholinesterase inhibitor)
48
management of myasthenia gravis?
- pyridostigmine
- immunosuppression: prednisolone initially (azathioprine, cyclosporine, mycophenolate mofetil may also be used)
- thymectomy
49
Ix of myasthenia gravis?
- single fibre electromyography: high sensitivity (92-100%)
- CT thorax to exclude thymoma
- CK normal
- autoantibodies
50
bromocriptine, ropinirole, cabergoline, apomorphine
dopamine agonists
| - for parkinsons
51
selegiline
MAO-B inhibitors
| - for parkinsons
52
entacapone, tolcapone
COM-T inhibitor
| - COMT is an enzyme involved in the breakdown of dopamine, and hence used as an adjunct to levodopa therapy
53
what class of drug is topiramate?
carbonic anhydrase inhibitor
54
most common autonomic symptoms seen in Guillain Barre?
tachycardia, urinary retention
55
LP in Guillain Barre - what findings?
rise in protein with a normal white blood cell count (albuminocytologic dissociation) - found in 66%
56
Nerve conduction studies in Guillain Barre - what findings?
- decreased motor nerve conduction velocity (due to demyelination)
- prolonged distal motor latency
- increased F wave latency
57
which parkinsonian medication is associated with pulmonary/ cardiac/ retroperitoneal fibrosis?
ergot-derived dopamine receptor agonists (bromocriptine, cabergoline)
58
features of multiple system atrophy?
- parkinsonism
- autonomic disturbance
- ----- erectile dysfunction: often an early feature
- ----- postural hypotension
- ----- atonic bladder
- cerebellar signs
59
Features of Neurofibromatosis type 1 (aka von Recklinghausen's syndrome)?
- Café-au-lait spots (>= 6, 15 mm in diameter)
- Axillary/groin freckles
- Peripheral neurofibromas
- Iris hamatomas (Lisch nodules) in > 90%
- Scoliosis
- Pheochromocytomas
60
what chromosome is affected in NF1?
chromosome 17
- gene mutation that affects neurofibromin
61
Features of neurofibromatosis type 2?
- Bilateral vestibular schwannomas
| - Multiple intracranial schwannomas, mengiomas and ependymomas
62
What chromosome is affected in NF2?
chromosome 22
63
management of migraines in pregnancy?
- 1st line paracetamol
- 2nd line: NSAIDs in the first and second trimester
AVOID aspirin and opioids e.g. codeine
64
what artery is affected in Lateral medullary syndrome?
posterior inferior cerebellar artery
65
features of Lateral medullary syndrome / Wallenburg syndrome?
Cerebellar features: ataxia, nystagmus
Brainstem features:
- ipsilateral: dysphagia, facial numbness, cranial nerve palsy e.g. Horner's
- contralateral: limb sensory loss
66
In patients with Guillain-Barre syndrome, respiratory function should be monitored with:
FVC (Force Vital Capacity) to monitor respiratory function
67
right congruous homonymous hemianopia with macula sparing.
| where is the lesion
LEFT occipital cortex (opposite side!)
68
contraindications for triptan use?
ischaemic heart disease or cerebrovascular disease
69
acute management of cluster headache?
s/c sumatriptan + 100% O2
70
Prophylaxis of cluster headache?
verapamil
71
Complications of aneurysmal SAH?
- Re-bleeding: usually in first 12h
- Vasospasm: typically 7-14 days after
- Hyponatraemia (SIADH)
- Seizures
- Hydrocephalus
- Death
72
1st line management of spasticity in multiple sclerosis?
gabapentin or baclofen.
73
management of neuroleptic malignant syndrome?
- stop antipsychotic
- IV Fluids
- dantrolene- directly relaxes muscles by inhibiting calcium release from the sarcoplasmic reticulum
- bromocriptine, dopamine agonist
74
MOA of ondansetron
5-HT3 (serotonin) antagonist
75
SEs of ondansetron
most common- constipation
| - Prolonged QT
76
Neurological features of Friedreich's ataxia
Gait ataxia + kyphoscoliosis are most common
- absent ankle jerks/extensor plantars
- cerebellar ataxia
- optic atrophy
- spinocerebellar tract degeneration
77
Associations of Friedrich's ataxia?
- HOCM (90%, most common cause of death)
- High arched palate
- diabetes mellitus
78
Features of Ataxic telangiectasia?
- Telangiectasia
- cerebellar ataxia
- IgA deficiency -> recurrent infections
- increased risk of leukaemia/ lymphomas
79
drug causes of gingival hyperplasia?
phenytoin,
ciclosporin,
calcium channel blockers (especially nifedipine)
80
Cutaneous features of tuberous sclerosis?
- epigmented '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)
- café-au-lait spots* may be seen
81
Management of Ramsay Hunt syndrome?
oral acyclovir and steroids
82
MOA of procyclidine?
antimuscarinic.
- help tremor and rigidity
- treats drug induced parkinonism
83
management of absence seizures in pregnancy?
lamotrigine
- ethosuximide / sodium valproate would not be first line in pregnancy/ breast feeding
84
Features of juvenile myoclonic epilepsy?
triad of:
- Myoclonic seizures
- Generalised tonic-clonic seizures
- Absence seizures
85
Management of juvenile myoclonic epilepsy?
sodium valproate
86
Lambert Eaton myasthenic disorder assoc w?
Small cell lung ca + less commonly- ovarian/ breast ca
87
anti-NMDA receptor encephalitis assoc w?
ovarian teratoma
88
Ix of anti-NMDA receptor encephalitis?
- anti-NMDA receptor antibodies
- US abdo for ovarian teratoma
- MRI head / CSF may be normal
89
Treatment of NMDA encephalitis?
- immunosuppression with IV steroids, Ig, rituximab, cyclophosphamide or plasma exchange, alone or in combination.
- Resection of teratoma
90
drug causes of tinnitus?
- Aspirin/NSAIDs
- Aminoglycosides - gentamicin
- Loop diuretics
- Quinine
91
CT findings in HSV encephalitis?
medial temporal and inferior frontal changes (e.g. petechial haemorrhages)
- normal in one-third of patients
92
inheritance pattern of Facioscapulohumeral muscular dystrophy
auto dom
93
what is juvenile myoclonic epilepsy classically triggered by?
- in the morning/following sleep deprivation
| - photosensitivity
94
What is parinaud syndrome?
- Upward gaze palsy, often manifesting as diplopia (can’t look up)
- Pupillary light-near dissociation (Pseudo-Argyll Robertson pupils)
- Convergence-retraction nystagmus
95
Where is the lesion in Parinaud syndrome (can't look up)?
Dorsal midbrain
- rostral interstitial nucleus of MLF controls vertical gaze
96
What infection can cause spastic paraparesis?
HIV -> transverse myelitis
97
features of conduction aphasia?
speech fluent but repetition is poor. aware of the mistakes they are making. comprehension is normal.
98
Where is the lesion in conduction aphasia?
arcuate fasiculus - the connection between Wernicke's and Broca's area
99
Features of global aphasia?
severe expressive and receptive aphasia, may still be able to communicate using gestures.
100
Features of wernicke's aphasia?
receptive aphasia
- Comprehension impaired.
- Speech fluent but makes no sense (Word salad)
101
where is the lesion in wernicke's aphasia?
superior temporal gyrus
- usually supplied by inferior division of Left MCA
102
features of Broca's aphasia?
Expressive aphasia.
- Speech is non fluent, laboured, halting.
- repetition impaired
- comprehension normal
103
Where is the lesion in Broca's aphasia?
Inferior frontal gyrus.
- typically supplied by superior division of the left MCA.
104
speech fluent but repetition poor. Comprehension intact.
Conduction aphasia
| - classically due to stroke affecting the arcuate fasciculus
105
What distinguishes between Neuromyelitis optica and Multiple sclerosis?
NMO-IgG seropositive
| - Antibodies against aquaporin 4 antigen
106
absolute criteria for diagnosis of neuromyelitis optica?
optic neuritis, acute myelitis
107
What is Neuromyelitis optica?
- aka Devic's disease
- autoimmune disorder attacking optic nerves and cervical cord
- vomiting is common
108
Diagnosis of neuromyelitis optica?
- requires bilateral optic neuritis, myelitis and 2/3 criteria:
1. spinal cord lesion involving >/=3 Spinal levels
2. Initially normal MRI brain
3. Aquaporin 4 positive serum antibody
109
what is the autoantibody in neuromyelitis optica?
NMO-IgG - aquaporin 4 positive antibody
110
Causes of bilateral facial nerve palsy?
- sarcoidosis
- Guillain-Barre syndrome
- Lyme disease
- bilateral acoustic neuromas (NF2)
- Bell's palsy (can be b/l)
111
Causes of LMN unilateral facial n palsy?
- bilateral causes
- Bell's palsy
- Ramsay-Hunt syndrome
- acoustic neuroma
- parotid tumours
- HIV
- multiple sclerosis*
- diabetes mellitus
112
Causes of UMN unilateral facial n palsy?
Stroke, MS
113
what medication does paroxysmal hemicranial and hemicrania continua respond well to?
indomethacin
114
SE of Lamotrigine?
Stevens-Johnson syndrome
115
what areas of the spinal cord are affected in subacute combined degeneration of the cord?
dorsal columns + lateral corticospinal tracts
116
features of subacute combined degeneration of the cord?
- joint position and vibration sense lost first then distal paraesthesia
- UMN signs typically develop in the legs, classically extensor plantars, brisk knee reflexes, absent ankle jerks
117
DVLA advice for Meniere's disease?
patients should inform the DVLA.
| cease driving until satisfactory control of symptoms is achieved
118
prevention of attacks in meniere's diesese?
betahistine and vestibular rehabilitation exercises
119
1st line for newly diagnosed Parkinson's who have motor symptoms affecting their quality of life?
Levodopa
120
management of Bells palsy if paralysis does not improve within 3 wks?
refer urgently to ENT
- referral to plastic surgery may be appropriate for patients with more long-standing weakness e.g. several months
121
Infections assoc w autonomic neuropathy?
HIV, Chagas' disease, neurosyphilis
122
what conditions in which one should start anti-epileptics after first seizure?
if any of the following present:
- neurological deficit
- brain imaging shows a structural abnormality
- EEG shows unequivocal epileptic activity
- patient or family/ carers consider the risk of having a further seizure unacceptable
123
features of restless legs syndrome?
- akathisia: uncontrollable urge to move legs
- paraesthesias e.g. 'crawling' or 'throbbing' sensations
- periodic limb movements of sleeps (PLMS)
124
Causes/ associations of restless legs sydnrome?
- idiopathic (50% have +ve FHx)
- iron deficiency anaemia
- uraemia
- diabetes mellitus
- pregnancy
125
Diagnosis of restless legs syndrome?
clinical, ferritin to rule out IDA
126
Management of restless legs syndrome?
- simple measures: walking, stretching, massaging affected limbs
- treat any iron deficiency
- 1st line: dopamine agonists (e.g. Pramipexole, ropinirole)
- benzodiazepines
- gabapentin
127
1st line medical management of restless legs syndrome?
dopamine agonists e.g. pramipexole, ropinirole
128
absence seizures: what is the probability of the patient becoming seizure-free during adolescence?
90-95%. good prognosis
129
dementia pugilistica?
- assoc w boxers
| - chronic traumatic encephalopathy
130
a scale that measures disability or dependence in activities of daily living in stroke patients
Barthel scale
131
features of medication overuse headache?
- present for 15 days or more per month
- developed or worsened whilst taking regular symptomatic medication
- highest risk: using opioids and triptans
- may be psychiatric co-morbidity
132
management of simple analgesics and triptans in medication overuse headache?
stop abruptly (may initially worsen headaches)
133
management of opioids in medication overuse headache?
gradually withdrawn
134
confusion, behavioural change, ataxia, hemiparesis and visual deficits. in pt on natalizumab/ other HIV or MS drugs
Progressive multifocal leukoencephalopathy
| - due to reactivation of JC virus
135
what is transverse myelitis?
inflammation of the spinal cord. can be uni/ bilateral. characterized by neurologic dysfunction in motor and sensory tracts.
-> causes UMN weakness in lower limbs
136
auditory agnosia - which lobe
temporal
137
Palatal myoclonus - where is the lesion
in the triangle of Guillain and Mollaret (triangle linking the inferior olivary nucleus, red nucleus and the contralateral dentate nucleus)
- specific feature of hypertrophic olivary degeneration
138
causes of Predominately sensory Peripheral neuropathy
- diabetes
- uraemia
- leprosy
- alcoholism
- vitamin B12 deficiency
- amyloidosis
139
causes of Predominately motor Peripheral neuropathy
- Guillain-Barre syndrome
- porphyria
- lead poisoning
- hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- diphtheria
140
causes of Demyelinating Peripheral neuropathy
- Guillain-Barre syndrome
- chronic inflammatory demyelinating polyneuropathy (CIDP)
- amiodarone
- hereditary sensorimotor neuropathies (HSMN) type I
- paraprotein neuropathy
141
causes of Peripheral neuropathy of axonal pathology
```
alcohol
diabetes mellitus*
vasculitis
vitamin B12 deficiency*
hereditary sensorimotor neuropathies (HSMN) type II
```
142
Gerstmann's syndrome: alexia, acalculia, finger agnosia, R-L disorientation --- where is the lesion?
Lesion of Dominant parietal
-> dominant = opposite of dominant hand
143
SE of Vigabatrin (AED)?
V for Visual field defects.
40% of patients develop visual field defects, which may be irreversible
visual fields should be checked every 6 months
144
genetics of Charcot-Marie-Tooth disease (hereditary sensorimotor neuropathy type I)
auto dom, defect in PMP-22 gene (which codes for myelin)
145
features of Charcot-Marie-Tooth disease (hereditary sensorimotor neuropathy type I)?
- features often start at puberty
- motor symptoms predominate
- distal muscle wasting, pes cavus, clawed toes
- foot drop, leg weakness often first features
146
what is myotonic dystrophy?
an inherited myopathy with features developing at 20-30 years old.
- affects skeletal, cardiac and smooth muscle.
- 2 main types: DM1 and DM2.
147
Genetics of myotonic dystrophy?
auto dom, trinucleotide repeat disorder
148
genetics of DM1 (Myotonic dystrophy type 1)
Chr 19, CTG repeat at the end of DMPK (Dystrophia Myotonica-Protein Kinase) gene
149
Genetics of DM2 (myotonic dystrophy type 2)?
Chr 3, repeat expansion of the ZNF9 gene
150
Difference between DM1 and DM2?
DM1: distal weakness more prominent
DM2: proximal weakness more prominent, severe congenital form not seen
151
General features of myotonic dystrophy?
- myotonic facies (long, 'haggard' appearance)
- frontal balding
- bilateral ptosis
- cataracts
- dysarthria
- myotonia (tonic spasm of muscle)
152
urinary incontinence + gait abnormality + dementia
normal pressure hydrocephalus
153
triad of features in normal pressure hydrocephalus?
- urinary incontinence
- dementia, bradyphenia (slowed thinking)
- gait abnormality (may be similar to Parkinson's disease)
154
Ix of Normal pressure hydrocephalus?
Imaging showing hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement
155
Mx of normal pressure hydrocephalus?
VP shunting.
156
what antibiotics may exacerbate myasthenia gravis?
gentamicin
- macrolides (clari/erythromycin), quinolone (ciprofloxacin), tetracyclines (doxy)
157
what are some drugs that may exacerbate myasthenia?
penicillamine, procainamide, BB, lithium, phenytoin, quinidine, antibiotics e.g. gentamicin
158
what class of drug are triptans?
5-HT1 agonists
159
CI for triptans?
history of, or RF for, ischaemic heart disease/ Cerebrovascular disease
160
features of CJD?
- dementia (rapid onset)
| - myoclonus (e.g. "notable jerk" usually provoked by startle)
161
Ix of CJD?
CSF usually normal
- EEG: Biphasic, high amplitude sharp waves (in sporadic CJD)
- MRI: hyper intense signals in the basal ganglia and thalamus
162
most common presenting features of new variant CJD?
usually younger patients (average age. = 25y)
| - anxiety, withdrawal, dysphonia
163
causes of miosis?
- Horner's syndrome
- Argyll-Robertson pupil
- senile miosis
- pontine haemorrhage
- congenital
164
what is pituitary apoplexy?
Sudden enlargement of a pituitary tumour (usually non-functioning macroadenoma) secondary to haemorrhage or infarction.
165
Features of pituitary apoplexy?
- sudden onset headache similar to that seen in subarachnoid haemorrhage
- vomiting
- neck stiffness
- visual field defects: classically bitemporal superior quadrantic defect
- extraocular nerve palsies
- features of pituitary insufficiency
e. g. hypotension/hyponatraemia secondary to hypoadrenalism
166
Diagnostic ix of pituitary apoplexy?
MRI
167
Mx of pituitary apoplexy?
- urgent steroid replacement due to loss of ACTH
- careful fluid balance
- Surgery
168
congruous vs incongruous visual field defect?
A congruous defect simply means complete or symmetrical visual field loss and conversely an incongruous defect is incomplete or asymmetric.
incongruous defects = optic tract lesion; congruous defects= optic radiation lesion or occipital cortex
169
what hormone levels are low in narcolepsy?
orexin (hypocretin)- a protein responsible for controlling appetite and sleep patterns
170
Ix of narcolepsy?
multiple sleep latency EEG
171
Mx of narcolepsy?
Daytime stimulants e.g. modafinil, and nighttime sodium oxybate
172
restless legs syndrome- single most important blood test?
ferritin - exclude IDA
173
haemorrhage disease of the newborn - which AED may cause this?
phenytoin
174
DMARDS in Multiple sclerosis?
1. Beta interferon - reduces relapse rates
2. glatiramer acetate - immunomodulating drug
3. natalizumab - antagonises Alpha-4 beta-1-integrin, inhibits migration of leucocytes across the BBB
4. fingolimod: sphingosine 1-phosphate receptor modulator
175
Froins syndrome?
xanthochromia, high protein level and marked coagulation of CSF
-> caused by meningeal irritation and CSF flow blockage by tumour mass/ abscess
176
features of multiple system atrophy?
- parkinsonism
- autonomic disturbance: erectile dysfunction: often an early feature, postural hypotension, atonic bladder
- cerebellar signs
177
What is CADASIL?
cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
- rare cause of multi-infarct dementia
- often present with migraine
178
genetics of CADASIL?
NOCTH3 gene
179
features of CADASIL?
usually presents with migraines in middle age, followed by recurrent TIAs/ strokes -> neuro-cognitive decline, psych problems and dementia.
MRI- multiple widespread hyper intense lesions in the white matter, basal ganglia, thalamus and pons
180
what antibody is implicated in peripheral neuropathy in breast cancer?
Purkinje cell antibody
181
what antibody is implicated in ocular opsoclonus-myoclonus (assoc w breast/ small cell lung ca)?
Anti-Ri antibody
182
what antibody is implicated in stiff person's syndrome/ diffuse hypertonia (assoc w breast, colorectal, small cell lung ca)?
Anti-GAD antibody
183
what antibody is implicated in cerebellar syndrome assoc w ovarian/ breast cancer?
anti-Yo antibody
184
what antibody is assoc with small cell lung ca and neuroblastomas and may cause sensory neuropathy (may be painful), cerebellar syndrome and encephalomyelitis?
anti-Hu antibody
185
Ix of wernicke's encephalopathy?
- decreased red cell transketolase
| - MRi brain
186
What is the most specific finding on brain MRI for this Wernicke's encephalopathy?
enhancement of the mamillary bodies due to petechial haemorrhages
187
what is the cardiac features associated with myotonic dystrophy?
heart block, cardiomyopathy
188
What AED is assoc with the rare SE of Steven Johnson syndrome?
lamotrigine
189
common peroneal nerve palsy?
foot drop, weakness of foot dorsiflexion, foot eversion, extensor hallucis longus
- sensory loss over the dorsum of the foot and lower lateral part of the leg
- wasting of the anterior tibial and peroneal muscles
190
features of acute disseminated encephalomyelitis?
- autoimmune demyelinating disease
- can occur following infection
- acute onset of multifocal neurological symptoms
191
mx of acute disseminated encephalomyelitis?
IV steroids, consideration of IVIG where this fails
192
1st line mx of restless legs syndrome?
dopamine agonists e.g. ropinirole, pramipexole
193
what kind of anaemia is phenytoin assoc w?
folate deficiency -> megaloblastic anaemia.
194
SEs of levodopa?
```
- dyskinesia
'- on-off' effect
- postural hypotension
- cardiac arrhythmias
- nausea & vomiting
- psychosis
- reddish discolouration of urine upon standing
```
195
1st line management of parkinsons disease if the motor symptoms are not affecting the patient's quality of life?
dopamine agonist (non-ergot derived) e.g. ropinirole, levodopa or monoamine oxidase B (MAO‑B) inhibitor e.g. selegiline
196
management of drug induced Parkinsonism?
antimuscarinics e.g. procyclidine, benzotropine, benzhexol (trihexyphenidyl)
197
what part of the hypothalamus is affected with a craniopharyngioma?
ventromedial area of the hypothalamus
198
management of benign essential tremor when propranolol is CI?
primidone
199
Lead poisoning vs acute intermittent porphyria?
both cause increase in urinary delta-aminolevulinic acid, only lead poisoning causes basophilic stippling
200
Features of nitrous oxide toxicity?
- predominantly sensory neuropathy and assoc myelopathy (leg weakness, increased AND decreased reflexes)
- similar to B12 deficiency as NO interacts w Vitamin B12 causing selective inhibition of methionine synthase
201
Features of orbital apex syndrome?
optic neuropathy, proptosis, Horner's syndrome, cheimoses (swelling of the ocular surface membranes), ophthalmoplegia, involvement of first branch of the trigeminal nerve.
202
Classic features of temporal lobe epilepsy?
ascending epigastric aura
olfactory + gustatory hallucinations,
ictal fear,
automatism: lip-smacking, chewing, swallowing + fiddling/ tapping
203
Most common cause of temporal lobe epilepsy?
hippocampal sclerosis
dx: MRI brain.
Tx: surgery
204
mx of primary cervical dystonia/ torticollis?
Botulinum toxin injection
205
What can you measure to discriminate between organic and psychogenic seizures?
serum prolactin levels.
most seizures cause rise in serum prolactin whereas psychogenic seizures do not.
206
what type of nystagmus is associated with difficulty in reading down a menu/ text? and is usually better when reading a menu at coffee shops (usually positioned above your head)
downbeat nystagmus
207
Diagnosis of TB meningitis?
CSF PCR
208
differences in reflexes in conus medullaris vs cauda equina syndrome?
Cauda equina: Both ankle/ knee jerks affected
Conus medullaris: Knee jerks preserved, ankle jerks affected
209
Mx of Tourette syndrome?
Risperidone
210
what are the 5 classic presentations of lacunar strokes?
1. pure motor hemiparesis
2. pure sensory
3. sensorimotor
4. ataxic hemiparesis
5. clumsy-hand dysarthria
211
What nerve conduction study finding is assoc w Axonal neuropathy??
reduced compound muscle action potential amplitude
212
What nerve conduction study finding is assoc with myelin disorders?
reduction in conduction velocity/ conduction block
213
features of inclusion body myositis?
- most common acquired myopathy in >50y, esp M.
- serum CK can be normal
- idiopathic inflammatory myopathy
- affects quads/ long finger flexors. often asymmetrical
214
cavernous sinus thrombosis affects which nerves?
CN 3, 4, 6, V1
215
Ipsilateral CNIII palsy + contralateral hemiparesis and hemiparkinsonism
Weber syndrome
| - occlusion of paramedian branches of the posterior cerebral artery
216
mx of miller-fisher syndrome?
IVIG +/- plasma exchange
217
SEs of topiramate?
weight loss + renal stones + cognitive / behavioural changes
218
Compression of anterior interosseous nerve / aka Kiloh-Nevin syndrome
- features?
pain in form + weakness of pincer movement of thumb and index finger
219
Parietal lesions in the dominant lobe can produce:
- disorders of language
- agnosia
- Gerstmann Syndrome (L-R confusion, acalculia, agraphia, finger agnosia)
220
Non dominant parietal lesions may cause:
- visuospatial dysfunction
- constructional apraxia (ie. clock face)
- anosognosia (lack of insight) and dressing apraxia
221
Triad of kearn sayers syndrome?
chronic progressive external ophthalmoplegia, pigmentary retinopathy and cardiac conduction defects
222
Diagnosis of kearn sayers?
muscle biopsy - characteristic 'ragged red fibres’ (due to increased number of mitochondria)
- often supplemented by PCR analysis of mitochondrial DNA for mutation detection
223
Presumed BZD overdose: management?
Supportive care
It is now advised that flumazenil should only be used to reverse benzodiazepines in anaesthesia. (Due to side effect profile)
224
Features of alcohol hallucinosis?
- psychosis of < 6 mo duration
- auditory hallucinations, often of persecutory or derogatory nature
- occurs in clear consciousness
225
Which antiepileptic exhibits autoinduction ie. return of seizures after 3-4 weeks of treatment?
Carbamazepine
226
Anterior uveitis: management?
topical corticosteroids and cycloplegic (mydriatic) drops E.g. atropine/ cyclopentolate
227
When should statins be started post acute stroke?
48 hours post-event
- potential for haemorrhagic transformation in early administration of statins.
228
Dawson fingers: often seen on FLAIR images - hyperintense lesions penpendicular to the corpus callosum
-> seen in multiple sclerosis
229
Drugs causing a peripheral neuropathy?
```
amiodarone
isoniazid
vincristine
nitrofurantoin
metronidazole
```
230
Acute dystonia: management?
Procyclidine (anticholinergic)
Usually occurs as an extra pyramidal side effect to dopamine antagonist
231
Gold standard imaging option to pick up brain tumour?
MRI brain with contrast
| - offers excellent resolution and can be used to distinguish between other pathologies (such as an abscess)
232
What kind of brain tumour may be associated with polycythaemia?
haemangioblastomas release erythropoietin so can cause a rise in haemoglobin
233
thunderclap headache with normal CT brain and normal or near-normal lumbar puncture results ?
Reversible cerebrovascular vasoconstriction syndrome (RCVS)
234
Reversible cerebrovascular vasoconstriction syndrome (RCVS) - diagnosis?
finding of diffuse arterial beading on angiography (CTA, MRA or catheter)
235
Reversible cerebrovascular vasoconstriction syndrome (RCVS): management?
no evidence-based treatments for RCVS
although bed-rest (avoid exercise) and nimodipine/verapamil are usually prescribed. manage hypertension
236
First line diagnostic test for delirium?
Confusion Assessment Method
237
MRI findings in CJD?
hyperintense signals in the basal ganglia and thalamus
238
Most sensitive investigation to diagnose myasthenia gravis?
Single fibre electromyography (92-100% sensitivity)
Serum ACh receptor antibodies (85% sensitivity)
239
Optic neuritis treatment?
IV methylprednisolone
- Optic neuritis treatment trial: increased speed of visual recovery and reduced risk of conversion to multiple sclerosis at 2 years compared to oral prednisolone
240
Parkinson’s dementia: management?
Rivastigmine
| - licensed treatment demonstrated in a randomised clinical trial
241
Contraindications to thrombolysis in stroke?
- stroke or traumatic brain injury in last 3 months
- previous intracranial haemorrhage
- seizure at onset of stroke
- intracranial neoplasm
- suspected subarachnoid haemorrhage
- LP in last 7 days
- GI Bleed in last 3 wks
- active bleeding
- pregnancy
- oesophageal actives
- uncontrolled hypertension >200/120
242
Hoarseness post thyroidectomy: how to investigate?
Hoarseness indicates recurrent laryngeal nerve damage.
1st line: laryngoscopy to assess for vocal cord paralysis and evaluate motility
243
acute disseminated encephalomyelitis?
post infectious encephalomyelitis
- autoimmune demyelinating disease of the CNS
e. g. post measles, mumps, rubella, varicella
tx: steroids +/- consideration of IVIg
244
what blood test might be raised in true seizure compared to pseudo seizure?
raised serum prolactin
245
features of Multifocal motor neuropathy?
acquired autoimmune demyelinating Moto neuropathy
- motor conduction block
- slowly progressive, distal motor neuropathy which progresses over many years
- usually normal sensation and reflexes
- asymmetric
246
multifocal motor neuropathy: what antibodies are usually raised?
Anti-GM1 antibodies
247
what is POEMS syndrome?
```
polyneuropathy
organomegaly
endocrinopathy
M-protein band from a plamacytoma
Skin pigmentation
```
248
most effective imaging modality for TIA?
diffusion-weighted MRI head which is very sensitive to small ischaemic lesions.
249
features of spinocerebellar ataxias?
- autosomal dominant
- progressive development of ataxia features e.g. gait disturbance, nystagmus, tremor
- usually begins between 30-40 yo
249
features of spinocerebellar ataxias?
- autosomal dominant
- progressive development of ataxia features e.g. gait disturbance, nystagmus, tremor
- usually begins between 30-40 yo
250
up to what percentage of individuals with Guillain Barre will experience long term weakness?
15%
251
what anti-epileptic drug may cause hyperammonaemic encephalopathy?
sodium valproate
252
management of valproate-induced hyperammonaemic encephalopathy?
L-carnitine
| - may reduce ammonia levels
253
HSV encephalitis LP findings?
Raised lymphocytes
| High protein
254
Prognosis of HSV encephalitis?
dependent on whether aciclovir is commenced early
> If started promptly mortality is 10-20%. > Left untreated mortality approaches 80
255
EEG findings of HSV encephalitis?
lateralised periodic discharges at 2 Hz
256
Myasthenia gravis:
| Situations in which thymectomy is generally not done?
```
when patients have
1. antibodies to MUSK
2. late onset disease
Or
3. purely ocular disease
```
257
Multiple sclerosis relapse rates in pregnancy?
relapse rates may reduce during pregnancy and may increase 3 to 6 months after childbirth before returning to pre-pregnancy rates
258
Chronic inflammatory demyelinating polyneuropathy: management?
Steroids and immunosuppressants
259
Management of off episodes in Parkinsons?
Can use subcut apomorphine to alleviate symptoms
260
ipsilateral optic atrophy and contralateral papilloedema
Foster- Kennedy syndrome
typically caused by a frontal lobe mass, normally a meningioma, which compresses on the ipsilateral optic nerve and olfactory nerve, while increasing intracranial pressure for the contralateral optic nerve sheath
261
Features of foster Kennedy syndrome?
optic atrophy + central scotoma in the ipsilateral eye
papilloedema in the contralateral eye
anosmia
262
neurological syndrome in which trivial trauma to a peripheral nerve e.g. sleeping on a limb, results in a mononeuropathy which may take weeks to resolve
Hereditary Neuropathy with Liability to Pressure Palsy (HNPP)
263
Ix of Hereditary Neuropathy with Liability to Pressure Palsy
deletion in the peripheral myelin protein 22 gene on chromosome 17
> autosomal dominant
264
treatment of hepatotoxicity or hyperammonemic encephalopathy in the context of an acute or chronic overdose of sodium valproate?
L-carnitine
> reduces serum ammonia levels
265
irregular low frequency tremor which is a combination of resting, postural and action tremor
+ hx of prev stroke
Holmes tremor
caused by a lesion in the red nucleus
Tx: levodopa / thalamotomy or chronic thalamic stimulation
266
Asymmetric bi-brachial motor weakness without sensory or bulbar involvement
+ multi focal areas of demyelination and motor block
Multi focal motor neuropathy with conduction block
| MMN-CB
267
Tx of multi focal motor neuropathy with conduction block?
| Acquired immune mediated demyelinating neuropathy
IV Ig
268
What electrolyte replacement can precipitate myasthenia crisis?
IV Mg
269
Multiple lung cysts in tuberous sclerosis?
Lymphangioleiomyomatosis
270
Difference in size criteria of cafe au lait spots to diagnose neurofibromatosis in children vs adults?
Children: >0.5cm
Adults: >1.5cm
271
Diagnosis of spontaneous intracranial hypotension?
MRI with gadolinium
> demonstrates distinctive dural gadolinium enhancement and downward displacement of brain on sagittal views.
272
Risk factors for spontaneous intracranial hypotension?
connective tissue disorders such as Marfan's syndrome
273
Key features of spontaneous intracranial hypotension?
strong postural relationship with the headache generally much worse when upright. Patients may, therefore, be bed-bound
274
Management of spontaneous intracranial hypotension?
usually conservative
| > if this fails an epidural blood patch may be tried
275
What MRI brain finding is highly specific for spontaneous intracranial hypotension?
MRI brain with contrast:
diffuse, smooth dural thickening with enhancement (in~80%).
others less specific but common:
cerebellar tonsillar descent, subdural fluid collections, decreased ventricular size, engorgement of the pituitary gland
276
What imaging should be considered in trigeminal neuralgia?
first line- contrast MRI brain: may detect compressing/ demyelinating cause in ~15%
MRA: in the other ~85%, MRA may identify neurovascular contact between a loop of the superior cerebellar artery and the trigeminal nerve -> causing displacement or atrophy
277
what other 2nd line medications to consider for treatment of trigeminal neuralgia after carbamazepine?
oxcarbazepine, baclofen, gabapentin, lamotrigine
for those refractory to two-drug trials: consider surgical intervention
278
prophylactic mx of cluster headaches?
verapamil, galcanezumab can reduce freq of cluster headaches
279
mx of SUNCT: short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing?
medically refractory, reports of response to lamotrigine
280
prophylaxis mx of tension type headaches?
amitriptyline
281
Most sensitive and specific test for prion proteins in the CSF to diagnose CJD?
real-time quaking-induced conversion assay
-uses fluorescent dye to detect prion protein present in the CSF
282
severely asymmetric Parkinsonism, fixed dystonia, myoclonus, cortical sensory deficits, apraxia, cognitive deficits ...?
corticobasal degeneration
- one of the Parkinson's plus syndromes
283
severely asymmetric Parkinsonism, fixed dystonia, myoclonus, cortical sensory deficits, apraxia, cognitive deficits ...?
corticobasal degeneration
- one of the Parkinson's plus syndromes
284
treatment for levodopa-induced dyskinesia?
amantadine
285
what autoantibody is positive in NMO?
serum aquaporin 4 antibody testing
286
management of intracranial hypotension?
epidural blood patch
287
Treatment options of tardive dyskinesia 2' anti-emetic, antipsychotic meds (dopamine antagonists)?
monoamine transporter 2 inhibitors e.g. valbenazine, deutetrabenazine, tetrabenazine
288
1st line treatment for MS relapses?
high dose glucocorticoids e.g. IV Methylpred (1g/d for 3-5 days)
289
2nd line treatment for MS relapses refractory to glucocorticoid treatment?
IM adenocorticotropin hormone gel, plasmapheresis
290
in Multiple sclerosis, what is defined as "activity"?
clinical relapses or MRI evidence of new or enlarging lesions
291
in Multiple sclerosis, what is defined as "activity"?
clinical relapses or MRI evidence of new or enlarging lesions
292
in Multiple sclerosis, what is defined as "progression"?
gradual accumulation of neurologic deficits independent of relapses
293
what monoclonal antibodies may be used for migraine prophylaxis?
erenumab, fremanezumab, eptinezumab, galcanezumab
293
in Multiple sclerosis, what is defined as "progression"?
gradual accumulation of neurologic deficits independent of relapses
294
what CSF finding is characteristic of Guillain Barre syndrome?
albuminocytologic dissociation (high protein level with normal/mildly elevated leucocyte count)
295
what are good first line AEDs to initiate in older adults?
lamotrigine, gabapentin, levetiracetam
296
first line treatment for essential tremors?
propranolol, primidone, topiramate
297
2nd line treatment for essential tremors?
ie. after trying propranolol, primidone, topiramate
clonazepam
298
treatment of refractory essential tremors?
deep brain stimulation and focused US thalamotomy
299
what prophylactic agents may be used in migraines?
- propranolol, timolol, metoprolol
- amitriptyline, venlafaxine
- topiramate
- sodium valproate
- monoclonal abs
300
what investigation is performed in drug resistant epilepsy to evaluate for candidacy for epilepsy surgery?
1st step: video EEG
2nd step: advanced brain imaging
