general neuro Flashcards

1
Q

ADEM - what’s the problem

A

AI disease: Type IV hypersensitivity: myelin-specific T cell reaction&raquo_space; inflammation + demyelination of brain and spinal cord

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2
Q

ADEM vs MS

A

ADEM = single event of demyelination as a child
MS = progressive recurrent bouts of demyelination in young adult

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3
Q

ADEM trigger

A

trigger unknown - ?protein molecular mimicry.
esp. flu, MMR, human herpes
esp. mycoplasma pneumoniae, beta-haemolytic strep

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4
Q

ADEM - key features

A

4-8yo
1-3 weeks post infection
systemic inflammation: fever, headache, n/v

POLYsymptomatic of demyelination:
- 80% motor deficits, weakness
- 50% ataxia
- 50% cranial nerves
- optic neuritis usually bilateral
- altered conscious state, seizures

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5
Q

ADEM - MRI and CSF

A

MRI:
bilateral, multifocal but asymmetric, and large in size
WM abnormalities 90% - subcortical&raquo_space; periventricular

CSF abnormal: pleiocytosis, inc protein, oligoclonal bands in 0-29%

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6
Q

outcomes with ADEM vs ATM

A

ADEM: 60-90% full recovery
some may have residual deficits
Risk of subsequent diagnosis of multiple sclerosis is low (2-10%)

ATM: 50% normal outcome, 80% normal-good
risk of MS diagnosis also around 10%

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7
Q

main spinal tracts

A
  1. corticospinal, descending motor info for voluntary movement
  2. dorsal column, ascending sensory info (pressure, vibration, fine touch, proprioception)
  3. spinothalamic, ascending sensory:
    - lateral = pain and temp
    - anterior = pressure and crude touch
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8
Q

transverse myelitis - key features

A
  • inflammatory demyelination of spinal cord, lesions usually span multiple segments but ‘transverse’ due to sensory impact
  • infection/AI trigger

MOTOR, SENSORY AND SPHINCTER DYSFUNCTION:
- bilateral changes: weakness + sensation + pain
- urinary retention common
- UMN signs

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9
Q

UMN vs LMN signs

A

UMN:
inc muscle tone (due to loss of descending inhibition)
weakness
spastic, rigid paralysis
hyperreflexia, positive Babinski

LMN:
lower tone (due to loss of innervation)
weakness
flaccid paralysis, hypotonia
hyporreflexia
muscle atrophy, fasciculations

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10
Q

Rx for ADEM/ATM

A

IV methylpred +/- IVIG

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11
Q

most sensitive sequence for ATM/ADEM for MRI

A

MRI brain + spine w/ gadolinium, axial T2 weighted

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12
Q

optic neuritis - key features

A

central scotoma
visual acuity loss
lose colour vision - esp red
PAINful
RAPD

a/w infection or vaccination

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13
Q

key causes of optic neuritis

A

MS
NMO
ADEM

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14
Q

bilateral severe or recurrent optic neuritis - think what?

A

MOG associated

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15
Q

outcomes for opitc neuritis, and risk for developing MS

A

recovery up to 2y post - 85%
overall risk MS = 30%
- highest in first 2y, with abnormal MRI, >12, CSF oligoclonal bands

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16
Q

NMO - key features

A
  • recurrent optic neuritis/myelitis
  • NMO Abs target aquaporin 4Ab - 91% specific for NMO
  • treat with immunosuppressive agents to prevent relapse
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17
Q

NMO vs MS

A

i. Recovery of visual and spinal cord function is generally not as compete after each episode
ii. ON is more frequently bilateral in NMO than MS

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18
Q

main disease modifying therapy for MS

A

IFN-beta

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19
Q

CSF of MS vs ADEM

A

MS = oligoclonal bands
ADEM = pleiocytosis

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20
Q

common organisms causing bacterial meningitis <2mo and >2mo

A

<2m: GBS, E.Coli, listeria
>2m: strep pneumo, neisseria, Hib

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21
Q

kernig vs brudzinski’s

A

kernig = hip flexed, knee flexed -> straighten leg&raquo_space; pain at the back of the leg

burdzinski’s: lying spine, then flex neck&raquo_space; involuntary hip/knee flexion

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22
Q

Rx for meningitis

A

<2mo: benpen + cefotax
>2mo: cefotax/ceftx, can give dexa

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23
Q

normal number of neuts/lymphocytes in CSF

A

<1mo: 0 neuts, 22 lymph
>1mo: 0 neuts, 5 lymph

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24
Q

most common viral cause of meningitis

A

enteroviruses e.g. coxackie, polio, echo

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25
most common ways CNS abscesses form
1. direct 50% = single abscess e.g. infection, trauma 2. haematogenous 50% = multiple abscesses in MCA distribution e.g. pulmonary/cv infections
26
most common organism for CNS abscess
strep 60% staph aureus 20%
27
Rx for CNS abscess
metro + cefotax/ceftx
28
Mx for stroke and time to do this
alteplase within 4.5h clot retrieval within 6h
29
mortality and outcome of childhood stroke
mortality 5-10% 50% long-term neuroimpairment 10-20% suffer recurrent strokes
30
signs / symptoms more suggestive of haemorrhagic vs ischaemic stroke
Ischaemic: Focal limb or facial weakness Visual or speech disturbance Limb incoordination or ataxia Haemorrhagic: Headache with other neurological signs or symptoms Altered mental state Signs of raised ICP
31
imaging modality for haemorrhagic vs ischaemic stroke
haemorrhagic: non con CT +/- CTA ischaemic: MRI+MRA first, if not then CT+CTA
32
initial supportive care measures in stroke
avoid hypotension, hypoglycaemia, hypothermia, NBM until safe swallow, IV fluids
33
most and 2nd most common cause of ischaemic stroke in kids
arteriopathy 50% e.g. moya mya cardiac disease 25% others haematological e.g. prothrombotic / sickle cell
34
most common territory affected for ischaemic stroke
MCA
35
exam q: drug causing cerebral sinovenous thrombosis
l-asparaginase
36
symptoms of mca stroke
contralat weakness face/arm contralat homonymous hemianopia contralat sensory loss global aphasia
37
what is todd paralysis
brief paralysis post seizure
38
what is PRES
hypertensive encephalopathy - seizures, confusion vision loss
39
positive movement disorders: athetosis vs dystonia vs chorea/myoclonus vs tics
-athetosis = slow, writhing, non-rhythmic. worse with movement. -dystonia = sustained, non-rhythmic, worse with movement -chorea (distal muscles), myoclonus (multiple groups) = rapid, nonsuppressible - tics = rapid, suppressible
40
chorea makes you think of what disorders
- RF (sydenham's) - huntington's - thyroid - lupus
41
NMS vs serotonin syndrome
both hot and autonomic problems serotonin syndrome - 24h onset, 24h resolution - hyperreflexia, tremor, myoclonus - treat: with benzo NMS - days to weaks onset and resolution - hyporeflexia, rigidity - treat: bromocriptine
42
wilson's disease = what kind of movement disorder
dystonia
43
types of tremor
1. static (PD) 2. postural (thyroid / physiological) 3. intention (cerebellar e.g. Wilson's)
44
key features of essential tremor in kids
- not at rest, mostly at end of activity - hands/forearms - FHx - respods to alcohol
45
tourette - key features
onset before 7y multiple motor and vocal tics, nearly every day a/w ADHD
46
lesch-nyan key features
X linked disorder of purine breakdown, leading to excessive uric acid – choreathetosis, spasticity, chorea, biting and aggressive behaviours
47
key features - acute cerebellar ataxia
Dx of exclusion 1-3yo, resolves in 2-3 weeks 2-3 weeks post viral sudden onset truncal ataxia, vomiting, speech problems, cerebellar signs
48
most common degenerative vs hereditary ataxia
degenerative: ataxia telangectasia hereditary: friedreich's
49
key features of friedreich's ataxia
- Triple GAA repeat in gene encoding for the mitochondrial protein frataxin > oxidative injury - 10y onset - ataxia lower > upper - dysarthria
50
main drugs causing IIH
tetracyclines, doxycycline, nitrofurantoin, isotretinoin
51
main Rx for IIH
acetazolamide
52
key features of acute necrotising encephalopathy
- triggered by virus - HH6/lnfluenza - high LFTs without NH3 rise - mostly Asian
53
red flags for headaches
Progressive chronic headaches Focal neurological symptoms under 6 yrs Headache/vomiting that wakes child or present on waking (symptoms of raised intracranial pressure (ICP)) Consistent location of recurrent headaches VP shunt Known systemic disorder
54
primary headache types
TTH: bilat, pressing, chronic non-progressive, stress, hours to days migraine: uni/bilat, PULSATILE, acute recurrent, worse with activity, aura, NV, photophobia cluster: unilat, around eye, autonomic Sx
55
most common migraine aura in kids
photopsia - flashes and floaters
56
Rx for headaches
acute: NSAID sumatriptan >12y chlorpromazine prophylactic: beta blocker (think of CI) cyproheptadine amitryptilline
57
CSF and MRI in AI encephalitis
CSF - pleiocytosis, inc protein MRI - non-diagnostic
58
Rx for encephalitis
IVIG + methylpred after 10 days if no response. >ritux
59
pathologenesis of anti-NMDA encephalitis
- IgG Ab to NR1 subunit of NMDA-rec > GABAergic pathways don't work
60
epidemiology of anti-NMDA encephalitis
- females - most common AI encephalitis
61
clinical presentation key features of anti-NMDA encephalitis
- prodrome in 50% - early phase: psych stuff, movement disorder, sleep disturbance, seizure - late phase: echolalia, autonomic, breathing badness - **40% females have ovarian teratoma**
62
outcome for anti-NMDA encephalitis
full recovery in 80% but up to 2years, very slow recovery releapse in 20%
63
what is opsoclonus
uncontrolled, irregular, and nonrhythmic eye movement without intersaccadic intervals
64
cerebellar encephalitides /opsoclonus-myoclonus = think WHAT for exams
50% have neuroblastoma bruh
65
rasmussen encephalitis - key features
only one hemisphere affected - functional hemispherectomy required progressive refractory seizures
66
the two abx we care about for AI encephalitis
anti-NMDA anti-VGKC
67
opsoclonus-myoclonus syndrome - key features
opsoclonus myoclonus ataxia - often misdiagnosed with acute cerebellar ataxia of childhood CD20 B cells in CSF paraneoplastic - esp neuroblastoma