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becky: neuro MSK > LECTURE 9: PEDIATRICS > Flashcards

LECTURE 9: PEDIATRICS Flashcards

1
Q

NS first appears at about ___ of gestation

A

21 days (before pt knows she is pregnant)

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2
Q

neural tube closure occurs at ___ days, from ___ to _____

A

23-25 days, anterior to posterior

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3
Q

neural tube (spinal cord and brain) starts at

A

21 days (closure)
24 days: fused

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4
Q

telencephalon forms

A

cerebral hemispheres

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5
Q

diencephalon

A

thalamus, GP, hypothalamus

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6
Q

mesencephalon becomes

A

midbrain

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7
Q

metencephalon forms

A

pons, cerebellum

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8
Q

myelencephalon

A

medulla

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9
Q

dysraphism etiology

A

unknown mainly
but prevention: folate!
risk factors; low SES (maternal nutritional status, prenatal care, teratogens (alcohol, retinoic acid, valproic acid)

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10
Q

seizure meds could cause

A

DYSRAPHISM
chemo meds (folate metabolism)

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11
Q

diagnosis of dysraphism

A

ultrasound
alpha-fetal protein

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12
Q

prevention of dysraphism

A

FOLATE

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13
Q

zika causes

A

ancephaly

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14
Q

dysraphism is a disorder of

A

neural tube closure (anterior neuropore open: cranial dysraphism: brain coming out with skull defect)

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15
Q

what is spinal dysraphism?

A

spina bifida (vertebral abnormality)

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16
Q

3 types of spina bifida

A

myelomeningocele: SC and meninges through defect
meningocele: dura and arachnoid herniation
occulta: vertebral arch defect only, 10% of pop (tuft of hair)

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17
Q

spina bifida occulta is usually

A

asymptomatic
skin abnormalities maybe over defect
L5-S1

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18
Q

myelomeningocele

A

B AND B DYSFUNCTION
SC stuff
often associated hydrocephalus
*Sx helps with infection risk (high risk of meningitis) but neuro deficits remain
*risk for tethered cord syndrome

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19
Q

Tethered Cord

A

when patient grows, spinal cord is stuck
LMN dysfunction (cauda equina)
*prevention of mvmt of conus medullaris
most common is unilateral one leg LMN

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20
Q

arnold-chiari malformation

A

(descended cerebellar tonsils-incidental tonsillar herniation)
less than 5 mm is not a problem, often not symptomatic

21
Q

type I AC malformation

A

cerebellar tonsils displaced > 6mm

22
Q

type II AC

A

associated myelomeningocele (syrinx in spinal cord)

23
Q

type III AC malformation

A

associated encephalocele

24
Q

what are symptoms of arnold chiari malformation?

A

hydrocephalus (poor prog) or later with cerebellar, medullary, cranial nerve signs (or headache)

25
Q

how to treat arnold chiari malformation

A

close myelomeningocele,
VP shunt for hydrocephalus
posterior fossa decompression

26
Q

dandy walker malformation is what

A

cerebellar vermis abnormality, large cyst in posterior fossa
*common hydrocephalus
*spastic diplegia
*mental retardation

27
Q

developmental delay is defined as

A

less than 70% developmental quotient
global delay: 2 or more domains

28
Q

what could mimic regression?

A
  1. misperception of attained milestone
  2. seizures
  3. spasticitiy
  4. movement disorders
  5. hydrocephalus
29
Q

What is down syndrome?

A

multi-organ disorder (congenital chromosomal abnormality of 3 copies of 21)

30
Q

neurocutaneous disorders

A

skin manifestations of neurological disorders
phakomatoses

31
Q

red flags

A

fisting past 3 months
rolling before 3 months, not rolling after 7
MORO after 6 months
ATNR after 7 months
not sitting after 9 months
not talking after 2 years
echolalia after 3 yrs
stutter after 4 yrs

HANDEDNESS BELOW 12 MONTHS OR ROLLING BEFORE 3 MONTHS = WEAKNESS

32
Q

important features of down syndrome

A

trisomy 21
*short, stubby digits
*small pelvis
*AA instability of c-spine
*hypotonia
*intellectual disability
*SEIZURES
*cardiac and GI defects

33
Q

important features of fragile x syndrome

A

most common chromosomal cause of intellect. disability
low IQ, ADHD, seizures
low mm tone and hyperextended

34
Q

what does TORCHS stand for

A

toxoplasmosis
other (zika)
rubella
cytomegalovirus
herpes simplex, HIV
syphilis
(infection causes of delay)

35
Q

what is CP?

A

group of permanent disorders of mvmt/posture
cause activity limit
non-progressive disturbances in fetal/infant brain

36
Q

what can cause CP

A

hypoxia (10%)
toxins
metabolic disturbances
infections TORCHS
genetic, traumatic, vascular

37
Q

what is most common type of CP?

A

spastic (85-90%)
*others include dyskinetic, ataxic, mixed

38
Q

spastic hemiplegia, diplegia, quadriplegia

A

hemiplegia: arms (MCA)
diplegia: scissoring, adductor spasm
quadriplegia: seizures, intellectual disability, MOST SEVERE

39
Q

dyskinetic CP

A

Basal ganglia lesion
can’t walk
chorea, athetosis, dystonia

40
Q

what is the rarest form of CP?

A

ataxia CP
*cerebellum
*truncal/gait ataxia
*not responsive to drugs/PT

41
Q

2 types of neurocutaneous disorders

A

neurofibromatosis
tuberous sclerosis

42
Q

2 types of neurofibromatosis

A

NF1: PNS (cafe au lait, neurofibromas, PNS, optic glioma)
NF2: CNS (acoustic neuromas, meningioma, gliomas)

*scoliosis, hypotonia, poor coordination
*hearing, vision, language, epilepsy
*ADHD
*pain
*hypertension
*cancers

43
Q

what is tuberous sclerosis?

A

ADominant
NS, skin, bones, retina, kidney
calcifications on brain and into ventricles
*skin macules, retina hemartomas, CNS (mental retardation and seizures)

44
Q

adrenoleukodystrophy

A

x-link recessive
boys, 8
normal, then vision loss, dementia, seizures, spastic gait, behavior changes

*adrenal failure (high VLCFA)

45
Q

metachromatic leukodystrophy

A

lacking arylsulfatase A enzyme
A recessive

*degen of Central and peripheral meylin
*gait, mental deterioration, seizures

46
Q

duchenne’s MD

A

3-5: DF, hip flex/ext weak, hyperlordotic and toe walking
calf pseudohypertrophy
*wheelchair by 9-12 years (20: respiratory weakness lead to death or ventilator)

47
Q

Becker MD

A

toe walking
pseudohypertrophy
less severe, normal IQ, rhabdo, heart probs, quad weak, cramps
*ambulatory until 30 ish
*dystrophin levels in mm!

48
Q

spinal mm atrophy levels 0-4

A

LOSS OF SMN1 protein, LMN looks like ALS

type 0: prenatal, die before 6 mo
type 1: never sit (before 6 mo, die before 2 years)
type 2: never stand (6-12 mo, die around 25)
type 3: never run (after 1 yr, normal life)
type 4: adult prox leg weakness

becky: neuro MSK (14 decks)

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