Neurology Flashcards
when do you see oligoclonal bands
whenever BBB is disrupted / intrathecal production of IgG
MS, Lyme disease, autoimmune disease, brain tumour, lymphoproliferative disease
not seen in normal people
very non specific finding
indications for CEA
70-99% if symptomatic but not disabling
50-69% if symptomatic
> 60% asymptomatic
ideally within 2/52 by a skilled surgeon with < 3% periop mortality
NOT RECOMMENDED
< 50% symptomatic
< 60% asymptomatic
carotid stenting
Myasthenia gravis AChR Ab vs MuSK Ab Rx difference
AchR Ab - can use steroids + IVIG or PLEX
MuSK Ab - steroids + PLEX works best; thymectomy no benefit; pyridostigmine little benefit
(MuSK Ab - increased likelihood of bulbar involvement, increased ICU admission, mestinon response usually poor, no benefit of thymectomy)
wernicke-korsakoff
ataxia
encephalopathy
oculomotor - resolve straight away with thiamine
korsakoff - amnesia
NF1
Café-au-lait spots (>= 6, 15 mm in diameter) Axillary/groin freckles Peripheral neurofibromas Iris: Lisch nodules in > 90% Scoliosis Pheochromocytomas chromosome 17 (17 letters!)
NF2
bilateral acoustic neuromas
chromsome 22
parkinsons disease
avoid dopamine antagonists
domperidone anti emetic of choice as only acts in periphery
Neuromyelitis Optica NMO Devic’s disease
NMO IgG to aquaporin 4 70% in foot processes of astrocytes
optic neuritis, myelitis 90% recur
a/w SLE sjogrens
long cord lesions; no brain lesions
ICU myopathy
a/w GCC, asthma
increased CK; reflexes absent or low
NCS: sensation - normal; motor - decreased
EMG: fibrillation
ICU neuropathy
a/w SIRS, reflexes absent or low
NCS: decreased motor and sensory amplitudes
EMG: late - fibrillation
axonal, normal CK
alzheimers disease
CSF: increased tau; decreased beta amyloid
multiple sclerosis
HLA DR2 confers 3-4 X increased risk
anti neuronal Abs
paraneoplastic
myasthenia gravis
MUSK receptor Abs - worse disease
ACh R Abs
10% thymic CA; thymic hyperplasia common
Rx: IVIg
CN3 lesion
pupil spared in DM
pupil fibres travel on outside of bundle so impaired with compression
Guillain Barre
AIDP demyelinating AMAN, AMSAN - axonal reflexes absent or low NCS: normal sensory (but paraesthesias) a/w campylobacter, dysautonomia, increased CSF protein GQ1b Abs
hunger centre
hypothalamus
MCA
superior division - frontal signs
inferior division - temperoparietal signs
lenticulostriate (perforator) - no cortical signs
brain lesions
cryptococcus - lesions
toxo - multiple ring enhancing lesions
neurocysticercus - focal lesions, focal presentation - seizure, h/a, stroke; multiple small lesions
echinococcus - hydatid - large singular well defined lesion
lamotrigine
risk of SJS; clearance slowed by use of valproate
carbamazepine
HLA B1502 a/w SJS
meningioma
dural tail on imaging
NF2 ch 22
hormone sensitive - more in women, obese
MSA
hot cross bun sign on imaging
autonomic features
neck flexion
6th decade
PSP
hummingbird sign on imaging
neck extension; early falls
7th decade
GBS intubation indications
VC < 20ml/kg or < 1L or fall of 30%
negative inspiratory force < 30cm H2O
severe oropharyngeal weakness
fatigue
demyelination
conduction slowing, block
axonal
decreased amplitude
INO
ipsi eye cannot adduct
contra eye has leading eye nystagmus
medial longitudinal fasciculus lesion
bilateral is pathognomonic of MS
lateral medullary syndrome
ipsi ataxia arm, leg contra pain temp arm leg; ipsi face ipsi horners CN 9, 10, 11 lesion PICA
Gerstmann syndrome
dominant parietal lobe RAAF right to left confusion agraphia acalculia finger agnosia
lost in space
non dominant parietal lobe neglect one side body anosognosia (dont realise deficit) autotopagnosia (dont recognise limb) visual field issues apraxia dysarthria
eye deviation
away from paresis if hemispheric
toward paresis if pontine lesion
HLA in MS
HLA DR2
3-4 times increased risk from baseline
EBV 2.3 X increased risk
risk 2nd attack in possible MS
biggest predictor is number of lesions on baseline MRI
Uhthoffs
very MS specific
may be related to change in Na channels with temperature
Lhermittes
non specific
dissemination in time MS
> 1 month
or simultaneous enhancing and non enhancing lesions on MRI
ADEM acute disseminated encephalomyelitis
1-4 weeks pos EBV, mycoplasma, strep pyogenes, CMV, rickettsiae, varicella
IVMP once infx ruled out
fingolimod
MS treatment - oral
S1P receptor modulator
prevents lymphocyte egress from LNs
selectively targets CCR7+ cells i.e. Th17
fampridine
MS treatment
improvs walking speed
closes K+ channels on demyelinated axons
enzymopathies
glycolytic defect - failure of rise in lactate
amino acid defects - failure of rise in ammonia
myotonic dystrophy
AD anticipation from maternal side CTG triplet repeat cataracts ptsis distal wasting, weakness cardiac conduction defects myotonia; frontal balding, low IQ, hypersomnolence,insulin resistance, hypogonadal, hypopit
dermatomyositis
more cancer risk esp ovarian OR 4.4
more dysphagia, more acute
polymyositis
cancer risk esp NHL 2.1
amyloid
extracellular
neurofibrillary tangles / tau
intracellular
PD Rx
DAs: pramipexole, apomorphine, bromocriptine, pergolide, cabergoline sleepiness - amantadine, selegiline hallucinations - clozapine, quetiapine nausea - ondansetron, domperidone confusion - rivastigmine, donepezil
Charcot Marie Tooth
motor and sensory neuropathy slowly progressive distal weakness, atrophy, los sof reflexes PMP22 gene duplication ch 17 uniform slowly on NCS
heriditory neuropathy with pressure palsies HNPP
microdeletion PMP22 ch 17
focal palsies post minor trauma
auto domt
pure motor neuropathy
MND, polio, lead, vincristine, amiodarone, dapsone
pure sensory neuropathy
DM, HIV, B12 def, amyloid, sarcoid, sjogrens, paraneoplastic, B6 intoxication
lamotrigine interactions
+ valproate = rash, increased valproate levels
+ OCP = poor lamotrigine efficacy
OK in pregnancy
carbamazepine interactions
induces warfarin metabolism
HLA B1502 DRESS SJS
+ phenytoin = rash
pain and temp
spinothalamic
cross almost immediately (1-2 segments above entry)
vibe
dorsal columns
cros in medulla
motor
corticospinal
cross in medulla
L4/5 lesion
L5 radiculopathy as roots emerges below the vertebrae but knocked off on way through
MND gene
c9orf72 24%
but 95% is sporadic
Rx riluzole
TIA ABCD2 score
A age > 60 1 BP > 140/90 1 Unilateral weak 2 speech 1 > 60mins 2; > 10 mins 1 DM admit if > 4; 8% risk stroke next 3/12 if > 6