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RC: Specialties Woo Hoo > Neurology > Flashcards

Neurology Flashcards

1
Q

When do infantile spasms occur?

A

< 1 y.o.
Peak: 4-6 mo.
NOT newborn

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2
Q

What is the EEG pattern for infantile spasm

A

Hypsarrhythmia

  • high amp
  • multiple spike
  • chaotic
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3
Q

How do you treat infantile spasm

A

Vigabatrin (AE: Retinal toxicity)

OR
ACTH (irritable, adrenal suppression)

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4
Q

What is the AE you worry about with vigabatrin?

A

Retinal toxicity

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5
Q

EEG appears chaotic. High amplitude background with multifocal spikes. Likely Dx?

A

Infantile Spasm

Hypsarrhythmia

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6
Q

List two conditions associated with infantile spasm?

A

TS

Down Syndrome

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7
Q

List 3 things on ddx for infantile spasm:

A
  • Benign Myoclonus of infancy (<6 mo; normal EEG)
  • Sandifer syndrome (don’t see cluster but AbN movement due to GERD)
  • Breath Holding Spells (6-6 yo; ~1min. LOC; cyanotic or pallid; link with Fe deficiency; 100% resolve by 8 y.o.)
  • Infantile Masturbation (F >M, pelvic rocking, adduct leg, pelvic pressure, autonomic like flush or sweat, distractible)
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8
Q

What is West Syndrome?

A

Infantile Spasm + Hypsaarhythmia + Developmental Regression

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9
Q

What is the definition of epilepsy?

A

min. 2 unprovoked seizures

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10
Q

What % of pop’n have epilepsy?

A

1%

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11
Q

if first unprovoked seizure- what do you do?

A

Sleep deprived EEG

MRI if focal symptom or P/E finding.

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12
Q

Describe childhood absence epilepsy?

A 
6-7 y.o.
absence +++/d
otherwise N
Tx: ethosuximide, VPA, lamotrigine
70% outgrow as teens
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13
Q

Preschool. Atypical absence + atonic + myoclonus. Behavioural issue when older. Often pmhx (+) neurocutaneous or brain injury.

A

Lennox-Gastaut Syndrome

Behav or IQ issue when older.

EEG: 1.5-2Hz SLOW spike-wave

Steroids
VPA etc.

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14
Q

Seizures + lang loss in 3-8 y.o. Epileptic Dx?

A

Landau-Kleffner Syndrome

  • acquired epileptic aphasia
  • partial and GTC
  • lang loss

Tx: VPA (or Ethosuximide)
+ ** Steroids (for lang)

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15
Q

Preteen. Myoclonic jerk in morning with no LOC. Toothbrush jerk away. FHX absence sz.

A

Juvenile Mycolonic Epilepsy

EEG: generalized polyspike

Tx: VPA
Lamotrigine

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16
Q

10 y.o. Falling asleep and then child run in with focal facial mvmt x 1-2 minute. Drooling. One per night. Normal LOC.

A

Benign Rolandic Epilepsy

No meds needed (Keppra if frequent, prolonged, or secondary gran mal associated)

Resolve by puberty

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17
Q

What is the EEG finding in benign rolandic epilepsy?

A

Centro Temporal Spikes

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18
Q

List two meds for seizures in kids < 2 y.o.

A

Phenobarb
AE: sedation, reversible acquisition of milestones

Levetiracetam (keppra; fatigue, dizzy, SJS/TEN)

** except spasm= vigabatrin (retinal toxicity) or ACTH

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19
Q

List two meds for seizures in kids > 2 y.o.

A

Levetiracetam (keppra)

Valproate (hair thinning, wt gain, bone health, tremor, hepatitis, teratogen

** except Lennox-Gastaut (preschool absence, myotonic, clonic)= steroids +

** except Landau-Kleffner (3-8 y.o. sz + lang loss)= steroids +

** except Childhood Absence Syn (peak 6-7)= Ethosuximide.

** except Benign Rolandic Epilepsy at night w/ face no meds. If need Keppra

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20
Q

T or F: you can use carbamazepine for absence or myoclonic sz

A

False

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21
Q

T or F: safe to give phenytoin IM

A

False.
- very toxic and cause necrosis

** FOSphenytoin can be given IM

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22
Q

T or F: you SHOULD NOT give VPA in with potential metabolic dx

A

TRUE

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23
Q

Generalized 3 Hz spike and wave d/c in EEG. Dx?

A

Childhood Absence Epilepsy

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24
Q

T or F: you generally outgrow childhood absence seizures by adolescence.

A

True

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25
Q

6 y.o. Recurrent staring. Learning problem. EEG 3 Hz spike and wave. What do you tell family

  • high chance of GTC sz
  • tx not needed b/c benign nature of condition
  • reasonable to start oxcarbazepine
  • likely outgrown by adolescence
A

Likely outgrow by adolescence.

  • GTC not common
  • Need to tx= Ethosuximide
  • DO NOT give carbamazepine as makes it worse
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26
Q

List the dx criteria for paediatric migraine:

A 
min. 5 attack
last 1-72 hr
H/a has 2 of:
- uni/bil lateral
- pulsating/throb
- mod-severe pain
- worse w/ activity

PLUS one of:
N/V
photo/phonophobia

Not better explained by another condition

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27
Q

How do you know if someone has used OTC enough to cause med overuse h/a?

A

3X per week

x 3 month

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28
Q

What are absolute indications for head imaging in pt with h/a?

A
  1. New neuro symp (focal neuro in h/a, focal neuro on aura, seizure, abN gait)
  2. new focal P/E
  3. papilledema

Other questionable:

  • sudden onset worst of life (subarachnoid)
  • wake from sleep
  • worst in am
  • am emesis
  • worse with cough (chair)
  • recent head injury
  • change in type of h/a
  • occiput location
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29
Q

What are non pharma measures you can recommend for migraines or non migraine h/a in kids?

A

Regular meals + sleep

Exercise

Avoid Caffeine

Limit OTC to < 3X per week

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30
Q

15 y.o. with migraine w/ aura. Healthy otherwise. Lifestyle optimized. P/E normal. Recommend:

  • propranolol
  • ibuprofen
  • flunarizine
  • sumatriptan
A

Flunarizine 1st line prevention migraine med.

Ca2+ channel blocker

Studied in kids

Well tolerated

Rare AE:

  • wt gain
  • low BP
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31
Q

What is Tourette syndrome often associated with:

A
  • OCD
  • Anxiety
  • ADHD
  • ODD
  • Learning Diff
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32
Q

T or F: spastic diplegia associated with B/L PVL.

A

True

  • prem
  • ofte not seen until 6 mo. where ppt with early hand preferencd
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33
Q

Floppy Baby. How can you tell between central versus peripheral cause?

A

Central:
- low LOC (not bright)
- Floppy but strong limb (if weak is axial like head lag)
- Normal or ++ DTR
+/- other neuro deficit (sz, abN movement)

Peripheral
- weak + floppy
BUT bright, crying etc.
- LOW DTR
- no other neuro deficit (i.e. sz, abN movement)
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34
Q

2 day old term with hypotonia. Signif head lag. Alert + crying. ABSENT DTR. Likely dx:

  • SMA
  • DMD
  • Myotonic dystrophy
  • Prader will.
A

Spinal muscular atrophy.

Myotonic dystrophy possible but reduced DTR not absent.

DMD not in infancy.

Prader Willi= central hypotonia.

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35
Q

What is trigonalocephaly. What tx if severe?

A

Trigonal cephaly
= Triangle head
= Metopic closed

If severe= craniotomy to remove mitotic suture

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36
Q

What is the most common craniosynostosis?

A

Scapho cephaly

  • Sagittal suture fused
  • long + narrow football
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37
Q

Most common reason for Sx in child born with myelomeningocele:

A

Hydrocephalus

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38
Q

Which anomaly is most associated with SC anomaly in newborn:

  • Anorectal
  • Arthrogryposis (contracture)
  • Malrotation
  • Dislocated hip
A

Anorectal Defect

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39
Q

Vascular malformation on upper face. What do you monitor for:

  • Hearing loss
  • Cerebral AVM
  • Glaucoma
A

Glaucoma

  • Skin= Port wine
  • Brain= Leptomeningeal Angioma
  • Eyes= Glaucoma
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40
Q

Baby with right facial droop. can wrinkle forehead. Able to close eye. Dx?

A

Asymmetric Crying Face

= Absence of depressor anguli iris muscle.
- cosmetic issue only

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41
Q

How can you tell asymmetric versus facial palsy from compression of facial n. (forceps)?

A

Asymmetric Crying Face:

  • eye and forehead unaffected.
  • only see with crying

Facial Palsy= generalized expression
- resolve 2-3 mo. on their own

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42
Q

How do you differentiate plagiocephaly from craniosynostosis?

A
  • AbN shape a birth
  • NO bald spot
  • plagio: ipsilateral face anteriorly displaced versus craniosyn. posterior displaced
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43
Q

What CT head finding is expected in child with facial nevus V1?

Name 2 other complications

A

Leptomeningeal capillary-venous malformation (Angioma)

Other:

  • Glaucoma
  • Other CNS findings: Seizure, hemiparesis, DD, stroke like episodes
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44
Q

Is sturge weber inheritable?

A

No

  • Sporadic mutation on GNAQ mutation
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45
Q

List 3 neurocutaneous syndromes?

A 
NF
TS
Sturge-Weber
Incontinentia pigmenta
PHACES
Ataxia- Telangiectasia
Von-Hippel Lindau
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46
Q

Child with infantile spasm. Hypo pigmented patch of skin. Underlying condition? What test to confirm this?

A

Tuberous Sclerosis

Genetic Testing (TSC1 or TSC2)

Or MRI

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47
Q

List Criteria for TS:

A

2 major or 1 major + 2 minor.

Listed Major…

“FAT SEGA RASH”

  • Fibroma: Ungal (min. 2), angiofriboma (min. 3)
  • Angiomyolipoma of kidney
  • Tubers (Cortical dysplasia)

SEGA

  • subependymal nodules
  • subependymal giant cell astrocytoma
  • Retinal hamartoma
  • Ashleaf spot (min. 3 min. 5 mm hypo pigment)
  • Shagreen patch (raised firm plaque)
  • Heart= rhabdomyoma

Minor= confetti skin, dental pits, intramural fibromas, multiple renal cyst etc.

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48
Q

How is TS inherited?

A

AD

w/ variable expression.

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49
Q

What is TS surveillance?

A

MRI q1-3 yr for SEGA

Renal image q1-3 yr

Neurodevelop testing

Watch for ICP, Sz

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50
Q

How many criteria do you need to dx NF1?

A

2 of 7 criteria.

“CAFEFOGS”

  • Cafe au lait
  • axillary or inguinal freckling
  • neurofibroma
  • Eye: lisch nodule
  • (+) FHX
  • optic glioma
  • Skeletal= sphenoid dysplasia, cortical thinning, pseudoarthroses
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51
Q

Name 3 traits of NF2

A

hearing

  • (+) FHX
  • Vestibular schwannoma, meningioma, glioma, neurofibroma etc.
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52
Q
  1. 5 y.o. F developing well. over 6 month regression. Consistent with:
    - TS
    - GM-1 gangliosidosis
    - Adrenoleukodystrophy
    - MELAS
A

GM-1 Gangliosidosis

  • neurodeg (ataxia, sz, regression)
  • HSM
  • cherry-red spot

Versus GM-2 Tay Sach= neurodeg < 1 and big head, cherry red spot

Versus Adrenoleukodystrophy degenerate by 3-4 y.o. but MALES. Adrenal insuff.

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53
Q

List 4 features of Rett Syndrome:

A
  • Developmental regression
  • acquired microcephaly (normal neo)
  • hypotonia
  • autistic like (lose eye contact, and mvmt)
  • lose hand skill, hand wringing
  • breath holding spells
  • sz
  • scoliosis
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54
Q

2 y.o. trunk writhing. flushed. diaphoretic. Grunt, rapid breathing. No LOC, can talk to distract, Next step:

  • refer EEG
  • GI to R/O GERD
  • Reassure
  • Refer psychologist
A

Reassure

= pleasurable behaviour similar to masturbation occur infancy onward

Masturbation may occur in girl 2-3 y.o.

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55
Q

Child in status. Unable to get IV. What do do:

  • Na nitroprusside
  • Rectal diazepam
  • Intubate
  • IM dilatin
A

Rectal diazepam

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56
Q

CPS statement. Sz management?

A

ABC (note bradycardia and low BP ominous signs)

  • 2 large bore IV
  • BG bedside

Pre hosp:

  • Ativan 0.1 mg/kg buccal (max 4)
  • diazepam 0.5mg/kg PR (max 20 mg)

IV:
- Ativan 0.1mg/kg IV (max 4 mg)

Still sz= repeat

Still sz=
IV: Fosphenytoin 20mg/kg
No IV: same IM

Infusion: midaz infusion

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57
Q

All can cause neonatal sz except:

  • ++ K
  • LO Na
  • LO Mg
  • LO Ca
  • alkalosis
A

++ K

alkalosis can cause decreased ionized Ca2+ due to increased binding of Ca2+ to albumin.

58
Q

T or F: metachromatic leukodystrophy has HSM

A

False.

59
Q

List 5 things on neonatal sz ddx.

A

DIMS:
Drugs > channelopathy (benign familial neonatal sz)

Infection
> Sepsis
> Meningitis
> Encephalitis

Metabolic
**> Low BG
> Low Ca
> Low Mg
> Low/High Na
> High Bili
**> IEM (a.a, galactosemia, urea cycle defect etc.)

Structural/ Sz/ Stroke
> ** HIE (60%)
> Focal hypoxia-ischemia (15%= **stroke)
> Intracranial **hemorrhage/ infarct (IVH, parenchymal, subarachnoid, subdural)
> Structural (lissencephaly, polymicrogyria)

60
Q

Best Med for absence seizures. Second option? What not to use?

A

Ethosuximide

Valproate

Lamotrigine

NO carbmazepine

61
Q

Febrile sz. What questions do you ask to assess risk of developing epilepsy?

Absolute risk of developing epilepsy?

If RF (+) risk of epilepsy?

A

Increased risk of epilepsy:

  • **FHX epilepsy
  • ** abnormal neurodevelopment
  • **complex febrile sz (focal, > 15 min., > 1 sz in 24)

Risk recurrent feb sz= 30-50%

Risk of epilepsy in general popn= 1%

Risk of epilepsy if typical febrile = 2%

Risk epilepsy if feb sz and (+) RF= 2-50%

62
Q

What is the typical age range for febrile seizures?

A

6 mo- 6 year

63
Q

T or F: risk of febrile seizure increased on day 1 of DTP vaccine and up to 2 week after MMR Vaccine

A

True

64
Q

List features of complex or atypical febrile seizures?

A

Focal
> 15 min duration
> 1 sz/ 24h

65
Q

T or F: you should work up a febrile sz?

A

False if typical.

LP if < 12 month, signs of meningitis.

66
Q

T or F: antipyretic will reduce risk of febrile sz

A

False.

67
Q

Kid w/ facial twitch in middle of night. Sz pattern on EEG?

A

Centro temporal

= Benign Rolandic Seizures

= No meds as resolve 2-4 yr from onset

68
Q

List AE for VPA and Phenytoin:

A

VPA

  • *- wt gain
  • hyper ammonia
  • *- tremor
  • *- alopecia
  • menses irregular
  • hepatic and pancreatic toxicity

Phenytoin

  • *- gingival hyperplasia
  • *- coarse facies
  • *- hirsutism
  • *- cerebellar (nystagmus, taxis)
  • SJS
  • Liver toxicity
69
Q

Phenytoin and VPA. Gums big can’t eat. Which med is responsible.

A

Phenytoin

AE:

  • gingival hyperplasia
  • coarse facies
  • hirsutism
  • cerebellar (nystagmus, taxis)
  • SJS
  • Liver toxicity
70
Q

What are night terrors?

A

2-7 y.o.
child terrified
unresponsive
worsen if try to console
Tx: reassure; outgrow by puberty
+/- scheduled awakening 15-30 min. than usual time x 2-4 week
RARE: If violent or injury consider benzo x fe months

71
Q

Child has frightening awakening, scream, cries. No recollection in am. Likely dx?

A

Night terror

72
Q

Most specific indication of sz in neonate:

  • high HR
  • irregular RR
  • high RR
  • abnormal eye mvmt
A

AbN eye mvmt

73
Q

When do you give pyridoxine for neo sz?

A

B6

Sz right after birth + RESISTANT to conventional tx

74
Q

Best treatment for tension h/a?

A

Simple analgesic (ibuprofen or acetaminophen)

+ Non-pharma

75
Q

Most frequent cause of school absence in teenage F:

  • h/a
  • dysmenorrhea
  • asthma
  • sore throat
A

Dysmenorrhea

76
Q

5 y.o. Constant h/a x 3 month. Worse. Impacting f’n. Dx to consider:

  • brain tumour
  • migraine
  • tension
  • behaviour
A

Brain tumour

77
Q

What age is sumatriptan approved for ?

A

Min. 12 y.o.

78
Q

List two periodic syndromes associated with migraine development as an adult.

A

Childhood periodic syn= occur in f with kids with migraine.
> GI
**- cyclic vomiting syn
**- abdo migraine (AP + N/V, pallor)

> Sleep

  • sleep walking
  • sleep talking
  • night terrors
79
Q

What is the most common cause of childhood h/a

A

Migraines

80
Q

List the layers of the brain (areas you think about when interpreting CT)

A 
Skin 
>Skull Bone
>Dura
>Arachnoid
> Subarachnoid Space
> Pia Layer
> Brain
81
Q

What is the shape of epidural bleed on CT?

A

FOOTBALL

  • arterial
  • assoc w/ skull #
  • brief LOC -> lucid -> then unconscious
82
Q

What is the shape of a subdural bleed?

A

CRESCENT

Football is E= epidural

Subdural- most common abusive head trauma
- gradual increase h/a and confusion

83
Q

How does a subarachnoid bleed look like on CT?

A

Within fissures

  • venous
  • traumatic or non-traumatic
84
Q

What type of brain injury is most commonly seen in abusive head trauma:

  • epidural
  • subdural
  • intraventricular hemm
A

Subdural

85
Q

T or F: Sickle cell stroke typically clinically seen.

A

False. Subclinical.

86
Q

GBS has been associated with the following infections?

  • E coli gastro
  • Shigella
  • Campylobacter
A

Campylobacter

GBS usually follow non-specific GI or resp 10d after

  • Campylobacter, Helicobacter pyloric
  • Mycoplasma
  • West Nile
  • Vaccine (rabies, flue, polio, Men C)
  • EBV, Lyme, CMV, H influ
87
Q

14 y.o. Duchenne. FVC and FEV1 fall. Likely complaint?

A

Morning H/A

Other:
daytime fatigue
daytime sleepiness

Point: nocturnal hypoventilation

88
Q

Dermatomyositis and Duchenne. Which help distinguish?

  • proximal muscle wk
  • rash on face + knuckle
  • abN muscle BW
  • onset < 5 y.o.
  • F >M
A

Dermatomyositis
F>M
Rash on face + knuckle
symmetric proximal weakness

BOTH onset usually after 5 y.o.

89
Q

T or F: If CK is normal - it can still be Duchenne.

A

False.

CK greatly elevated even at birth.

90
Q

How do you dx Duchenne?

A

Blood PCR for dystrophin gene.

OR
Muscle Bx
- connective tissue proliferation, degenerating and regenerating myofibers, inflammatory cell infiltrate

91
Q

How do you tx Duchenne

A

Steroids (keep prolong ambulatory, may improve long-term prognosis)

92
Q

List 3 comorbidities with Duchenne?

A

Cardiomyopathy
Scoliosis
Intellectual impairment (in all pt)
Depression

93
Q

Bilateral proximal weakness. Dysphagia, b/l ptosis. Upward gaze. Slow onset. CK normal. TSH normal. Give Dx.

A

Myasthenia Gravis

= rapid fatiguability of striated muscle.
- AI, familial, congenital, toxin induced (botulism)

Earliest sign: ptosis, EOM, dysphagia
- RAPID fatigue
- ** EMG = decremental response to repetitive stem
Tx: cholinesterase inhibitors (Neostigmine)

94
Q

What is dx of Duchenne on bx? What do you want to know to help with genetic counselling?

A

Lack of dystrophin.

What we want to know:

  • Mom a carrier?
  • Planning more kids?
  • Mom have sib and they want kids?
95
Q

4 complications of Duchenne:

A
  1. gross motor delay
  2. obesity
  3. cardiomyopathy
  4. restrictive lung dx
  5. scoliosis
  6. contracture
  7. steroid AE
96
Q

Hypotonic. Inverted V shaped mouth. Distal muscle wasting. Myotonia (slow relax after contraction like grip). Cataract. Hypothyroid common. CK high.. Dx and testing?

A

Myotonic Dystrophy

Dx: DNA analysis (expansion of CTG)

97
Q

Child with Duchenne. What is best test:

  • serum dystrophin assay
  • bx
  • molecular study
  • EMG
A

Bx

CC: hip girdle weakness by 2 y.o.
CK ++
Dx: PCR dystrophin gene mutation
If normal= Bx

98
Q

When an Duchenne be seen in a F?

A

If they are Turner’s (XO)

99
Q

List 3 complications of myotonic dystrophy

A

Weakness (initial distal then proximal; braces needed)

Cataracts

Speech difficulty

Altered Cardiac (heart block)

Endo: low thyroid, adrenal insuff, T1DM

GI: constipation, slow emptying

Low IgG

Increase CA risk

100
Q

Post traumatic pain. Burning. Allodynia. Neuropathic Pain. Tx?

A

Gabapentin

Other option: TCA (amitriptyline), Lamotrigine

101
Q

What is Erb’s Palsy?

A

C5+ C6

Shoulder adduct, internal rotation, elbow extended with finger flexed (waiter’s tip)

Recover > 80%

102
Q

What is Brachial Plexus Injury RF:

A

Multiparous mom
Shoulder dystocia
LGA
IDM

Protective: Twins, triplets, C/S

103
Q

What is Klumpke’s palsy

A

C8-T1
“Claw hand”
Usually associated w/ underlying anatomic abN or complete plexus injury

104
Q

Which is true about Erb’s palsy?

  • extension at wrist
  • preserved grasp
  • symmetric moro
A

Preserved Grasp

Extension at Elbow
Wrist + finger flexed

105
Q

Define cerebral palsy:

A

Non progressive in fetal or infant brain
Permanent
Disorder of mvmt

106
Q

Describe:

  • Spastic diplegia
  • Spastic quad
  • Hemiplegia
  • Extrapyramidal (athetoid, dyskinetic)
A

Hemiplegia: Unilateral

  • **infarct (stroke, IVH, malformation)
  • **hand preference
  • seizure, cognitive issue
Diplegia:
B/L
- **PVL, cyst, big ventricle (prem)
If term: ischemia, infection
- commando crawl, scissor
- normal intellect

Quad: whole body

  • Same etiology as diplegia
    • swallowing
  • IQ issue + sz

Extrapyramidal (athetoid, dyskinetic)

    • birth asphyxia
  • low tone > high tone > rigid > posturing
  • feeding issue, drooling
107
Q

T or F: red flag for ATNR - fixed x 30 sec or while crying or > 6 mo. age

A

True

108
Q

How can you help prevent CP

A

Maternal MgSO4 if < 32 wk

Cooling term if x 3d if HIE

109
Q

PVL and CP. What would you see on CT?

A

PVL = NECROTIC lesions in PV white matter.

110
Q

3 signs/symp that might confirm CP.

A
  • Spasticity
  • Scissoring
  • Brisk DTR
  • Difficulty swallowing
  • Developmental regression
111
Q

What management do you recommend for CP?

A 
MRI for pathology
No Routine BW
Multi-D (PT, OT +/- SLP, rehab team if severe)
Contracture
- passive stretch
- bracing
- botox
- baclofen
- rarely benzo
112
Q

List 5 RF or CP:

A

Antenatal:

  • prem
  • low BW
  • IUGR

Perinatal

  • birth asphyxia
  • complicated delivery

Post natal

  • abuse
  • trauma
  • meningitis/ encephalitis
  • cardiopul arrest
113
Q

What pop’n affect breath holding spell. What are the 2 types?

A

6 month- < 6 y.o.

Pallid: vasovagal -> anoxic

Cyanotic: cry -> apnea -> shunt -> cyanosis x < 15 second + LOC

114
Q

If you have breath holding spells what possible contributor must you R/O?

A

Iron deficiency anemia.

115
Q

How do you manage breath holding spells?

A
  • try to intercede before distress
  • ignore breath holding behaviour if start
  • educate on CPR, sz management
  • if no response consider mental health referral
116
Q

T or F: you are at increased risk of epilepsy if you had a sz with a breathing holding spell.

A

False

If recurrent anoxic sz can treat with anticholinergic like atropine.

117
Q

T or F: breath holding spells are typically self-limited.

A

True

Grow out within few years.
Can last until 5 y.o.

118
Q

What is atypical GBS (Miller Fisher Syn)?

A

Miller Fisher Syn
= external ophthalmoplegia
= ataxia
= areflexia

Papilledema can occur.

119
Q

Girl w/ acne with pseudo tumour cerebri. Likely etiologic drug?

  • OCP
  • Tetracycline
  • topical tretinoin
  • linda
  • EMG
A

tetracycline.

120
Q

List aetiologies for idiopathic intracranial HTN.

A
  • excess fat-soluble compound (**minocyline, metabolic dx)
  • infection (GBS, sinusitis, chronic otitis)
  • Drugs (**doxycycline, tetracycline, **isotretinoin)
  • renal (nephrotic)
  • heme (**polycythemia)
  • connective tissue (**antiphosphlipid)
  • endo (menarche, PCOS, hypothyroid)
121
Q

T or F: Idiopathic Intracranial HTN has normal CSF.

A

Yes

BUT high ICP

122
Q

What test must you do if you suspect increased ICP:

A

MRI + MRV

then LP

123
Q

How do you treat idiopathic increased HTN?

A

Tx underlying cause
+/- Serial LP
+/- acetazolamide (diamox)

124
Q

6 y.o. hx diplopia, h/a, ataxia. Where i lesion? What are two most likely brain tumour?

A

Posterior Fossa Tumour

Likely :

  • cerebellar astrocytoma
  • medulloblastoma
125
Q

Child with ataxia, diplopia, head tilt. Most likely:

  • Craniopharyngioma
  • Brainstem glioma
  • Cerebellar astrocytoma
  • Posterior fossa hemm
A

Brainstem glioma

  • Cerebellar astrocytoma= limb ataxia, h/a, vomiting
  • Brainstem glioma: diplopia, gait Cranial nerve.
  • Crania pharyngioma: endo abnormalities (Growth failure, delayed puberty, panhypopit)
  • posterior fossa hematoma: h/a, vomit, dizzy
126
Q

What are signs of cerebral herniation:

A

HTN + low HR + irregular breath

pupillary dilation

extensor posturing

127
Q

severe h/a collapse with progressive posturing. After intubation what do you do?

  • urgent CT
  • mannitol
A

Either…

Mannitol b/c med emergency and want to R/O reversible causes.

128
Q

Facial tics. What support dx of Tourette:

  • tic x 6 mon.
  • fhx of tic
  • ADHD
A

FHX of tics!

129
Q

Choreiform movement. Difficulty writing. Emotional lability. Throat swab normal. Likely dx?

  • huntington
  • sydenham’s
  • lupus
A

Sydenham’s chorea

130
Q

What treatment do you provide for stereotypic movement (like hand banging)

A
  1. Reassure
  2. Avoid trigger
  3. Don’t scold
  4. Ensure not ASD
131
Q

4 absolute contraindication to LP:

A
  1. Cardioresp instability
  2. Possible high ICP (mass, herniation)
  3. Infection over targeted site
  4. Bleeding (plt < 20, DIC)
  5. Spinal anomalies
132
Q

Child presents w/ ataxia and inability to sit up 2 wk after chicken box. What is dx?

A
  • most common acquired cause
  • post infectious, AI cerebellar demyelinated
  • 2-4 y.o.
  • common viral (varicella, post vaccine MMR)
  • NO FEVER
  • brain imaging normal
  • improve within 1 week and full recovery in 3 mon
133
Q

How do you differentiate post infectious acute cerebellar ataxia from meningoencephalitis?

A
  1. normal LOC (no encephalopathy)
  2. No meningeal signs
  3. Lack systemic (fever, tachy HR)
  4. W/U: MRI normal, CSF may be helpful (no pleocytosis)
134
Q

What are the top two causes of acute ataxia:

A
  1. post infectious cerebellar ataxia

2. drug ingestion

135
Q

Most common episodic and chronic ataxia?

A

Metabolic or genetic

136
Q

List a ddx for ataxia:

A 
Infectious
AI
Toxin
Mass (tumour, vascular, abscess)
Hydrocephalus
Trauma (post concussion syn)
Stroke
Sensory ataxia (GBS)
137
Q

Tonic movement. Ataxia, can’t close mouth. Started after mother gave suppository. Treatment:

  • rectal diazepam
  • nothing
  • diphenhydramine
  • phenobarb
A

EPS

= Diphenhydramine

138
Q

AE: hepatotoxic, pancreatitis, low plt. Which drug:

  • phenytoin
  • carbamazepine
  • pheobarb
  • VPA
A

VPA

139
Q

Why is diazepam not used as anticonvulsant in neonates?

A

LESS Liver metabolism

140
Q

On dilantin. Started septra for an infection. Return ataxia and abN speech. What mechanism?

  • septra UP dilantin absorption
  • septra displace dilatin from protein binding sites
  • septra causes LESS metabolism of dilantin
  • septra causes LESS excretion of dilantin
A

LESS metabolism of dilantin via septra

141
Q

Risk of recurrent sz within 1 year if afebrile GTC?

A

25%

RC: Specialties Woo Hoo (31 decks)

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