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H - pediatrics > Neuro > Flashcards

Neuro Flashcards

1
Q

Most common cause of status epilepticus

A

Prolonged febrile seizure (23-30%)

Acute symptomatic (eg CNS infection, AE drug overdose or non-adherence, metabolic derangement) 17-52% for whole category

Remote (cerebral migration disorders, dysgenesis, hie, neurodegenerative disorder) 5-19%

CPS statement in status epilepticus

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2
Q

Leukodystrophy history

A

history of loss of milestones or intellectual decline, gait disorder, signs of spasticity

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3
Q

most common leukodystrophy

A

adenoleukodystrophy: X-linked, presents age 5-8 years with cognitive impairment, spasticity and seizures
elevation of VLCFA accumulation

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4
Q

MRI in ALD

A

demyelination in occipital region that progresses anteriorly

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5
Q

Leukodystrophy + macrocephaly

A 
Canavan disease (spongy deterioration of the cerebral white matter) AR in ashk jew
Alexander disease
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6
Q

tay sachs disease

A

lysosomal storage disease - loss of milestones, macrocephaly, cherry red spots

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7
Q

MELAS

A

Mitochondrial encephalopathy lactic acidosis and stroke-like episodes

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8
Q

MERRF

A

myoclonic epilepsy with ragged-red fibers

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9
Q

Lesch-Nyhan syndrome

A

disorder of purine metabolism
hyperuricemia, spasticity, choreoathetosis, self-injurious behaviour
X-linked, mutation in the enzyme HGPRT

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10
Q

Wilson disease

A

defet in copper transportating protein causing liver cirrhosis and depostis of copper in basal ganglia –> EPS symptoms (dystonia and chorea). AR inheritence. Low ceruloplamin, high Cu

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11
Q

Menkes disease

A

defect in Cu transport causing deficiencies in Cu-containing enzymes –> grey matter degeneration, seizures and kinky hair. low serum Cu

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12
Q

Rett syndrome mutation

A

MeCP2

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13
Q

syndromes associated with craniosynostosis

A

apert, crouzon, pfeiffer

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14
Q

classifications of CP

A

spastic
dyskinetic (dystonic or choreoathetoid)
ataxic-hypotoic

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15
Q

Duane syndrome

A

Failure of normal development of the abducens nerve (CN6) –> LR muscle, impaired abduction of the eye

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16
Q

Moebius syndrome

A

bifacial weakness and abnormal eye movements from abnormal development of CN nuclei (6 and 7 usually)

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17
Q

AED for partial seizures

A

carbamazepine, oxcarbazepine

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18
Q

AED for generalized seizures

A

VPA, Lamotrigine, topiramate, levetiracetam

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19
Q

Lamotrigine indications and side effects

A

Generalized and absence
Interacts with VPA, so use low dose

SJS - increase dose slowly

20
Q

Topiramate indications and side effects

A

Generalized and partial

Acute angle closure glaucoma, sweating, cognitive dulling, weight LOSS

21
Q

Phenobarbital indications and side effects

A

neonatal seizures

cognitive effects, transition to different AED before 12 months

22
Q

Phenytoin side effects

A

cumulative toxicity - cerebellar atrophy

Gingival hypertrophy, fetal hydantoin syndrome

23
Q

VPA indications and side effects

A

Generalized with spike wave appearance, absence
–> mood stabilizer
liver failure/hepatitis, pancreatitis, hyperactivity, thrombocytopenia, rash, increase ammonia, weight GAIN, teratogenic

24
Q

Carbamazepine indications and side effects

A

partial, Rolandic
Worsens generalized sz, autometabolises so levels drift down with time
Leukopenia, aplastic anemia (CBC)
LFTs, thyroid function

25
Q

Oxcarbazepine indications and side effects

A

Partial

Hyponatremia, but no CBC changes (unlike CBZ)!

26
Q

Ethosuxamide

A

Absence

common: GI upset
rare: liver, bone marrow (no routine screening needed)

27
Q

Levatiracetam (keppra) indications and side effects

A

Used for many seizure types
Excreted by kidney, reaches therapeutic dose quickly
Main s/e is behaviour/irritability, which limits its use if present

28
Q

Idiosyncratic adverse effects of AEDs

A

rare, serious, life threatening - dose doesn’t matter, usually happens in first 6 weeks

29
Q

Dose related side effects of AEDs

A

common, mild: GI, HA, drowsy –> decrease dose by 25%

30
Q

Neonatal myasthenia gravis diagnostic test

A

Edrophonium (tensilon) test (BAChase inhibitor, brief improvement in symptoms)

31
Q

Neonatal myasthenia gravis presentation

A

hypotonia, poor feeding, newborn, normal pupils normal DTR, resolves

32
Q

Congenital mysthenic syndrome

A

Not autoimmune, inherited neuromusc disorder at NMJ with Ach pre/post but not because of antibodies = different treatment
Presents at different ages

33
Q

Congenital myotonic dystrophy

A

Mom 50%, presents at birth
More severe than neonatal myasthenia gravis - poor suck, decr GI motility, resp distress, decr fetal mvmts/polyhydramnios

34
Q

Congenital myotonic dystrophy mutation and inheritance

A

Triplet repeat Chromosome 19, AD

35
Q

DMD diagnostic test and biopsy findings

A

lack dystrophin with muscle fiber atrophy/necrosis

PCR for dystrophin gene mutation done first, if negative –> muscle biopsy

36
Q

DMD inheritance

A

2/3 X linked recessive, 1/3 spont mutation

37
Q

Guillain Barre Syndrome CSF findings

A

^^protein (2x) with normal Glu and NORMAL WBC (normally if ^prot then ^WBC)

38
Q

Guillain Barre Syndrome investigations

A

Motor nerve conduction velocity decreased, EMG: acute denervation of muscle, CK normal or increased

39
Q

Guillain Barre Syndrome poor prognostic factors (3)

A

CN involvement, intubation, max disability at presentation

40
Q

Guillain Barre Syndrome treatment

A

supportive, if rapidly evolving can do IVIG x 2.5 days, steroid not effective, spontaneous recovery

41
Q

IVH screening in prems

A

<32 weeks at 2nd week of life + 6 weeks, critically ill –> DOL 3
All at risk will have f/u at term (36-40) to look for PVL

42
Q

When does neural tube close?

A

4th week!

43
Q

Causes of Bell’s palsy (4)

A 
Post-infectious:
viral illness
Ramsey hunt syndrome (VZV or herpes - look for vesicles in ear canal)
Lyme disease
AOM
44
Q

Sturge-Weber Syndrome features

A 
facial port wine stain (V1)
leptomeningeal angioma
Glaucoma
Calcified vessels on head CT
Seizures in 80%
45
Q

Contraindications to ketogenic diet

A

fatty acid oxidation defects

carnitine deficiency

H - pediatrics (15 decks)

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