neuro 25 Flashcards

(43 cards)

1
Q

risk factors for CP

A

hypoxic ischemic insult to the brain in prenatal period, prematurity, low birth weight, chorioamnionitis, prenatal viral infections and prenatal stroke

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2
Q

hemiparetic CP

A

weakness and spasticity on one side of the body; early hand preference, fisting, and incr reflexes with an upgoing toe on the affected side

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3
Q

diparetic CP

A

spasticity of all four limbs, but affecting the legs more than the arms; normal intelligence and less likely to have seizures than other forms of CP

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4
Q

spastic quadriplegia

A

all four limbs affected; seizures often occur in the first 48 hrs of life

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5
Q

clinical manifestations of CP

A

flaccid weakness, asymm limb movements, or seizures; older kids get spasticity, dystonia, developmental delay, and drooling

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6
Q

key clue that a neuro disorder is CP

A

static; that is, not progressive

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7
Q

what intelligence tests can be used to establish mental retardation?

A

Stanford-Binet IQ and the Wechsler Preschool and Primary Scale of Intelligence

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8
Q

Profound mental retard

A

less than 25

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9
Q

Severe mental reated

A

25 to 40

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10
Q

Moderate mental retard

A

40-55

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11
Q

Mild mental retard

A

55-70

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12
Q

moro reflex

A

elicited by head extension; causes abduction of arms and leg extension, followed by slower abduction of arms; appears at birth and disappears at 3 months

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13
Q

tonic neck reflex

A

turning head and then arm and leg extended on the side of the turn; aka fencer; appears at 1 month and disappears at 5 mos

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14
Q

traction response reflex

A

lift baby by traction in both hands; head lag after 6 mos is pathologic and indicates hypotonia

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15
Q

parachute reflex

A

appears at 6 mos and persist; plunge infant downwards and he should thrust forward symm as if breaking fall

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16
Q

Rett’s syndrome

A

progressive neurodevelopmental disorder; generally affects girls only; normal development until 6-18 mos; stereotyped hand movements

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17
Q

two important peroxisomal disorders that can cause pediatric neuro issues

A

X-linked adrenoleukodystrophy and Zellweger’s syndome

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18
Q

neurodegerantive diseases involving the white matter

A

metachromatic leukodystrophy, Krabbe disease, adrenoleukodystrophy, Pelizaeus-Merzbacher disease, Canavan disease, and Alexander disease

19
Q

congenital macular cherry-red spots found in what

A

Tay-Sachs, Sandhoff disease, Niemann-Pick disease, Gaucher disease, metachromatic leukodystrophy, and the sialidoses

20
Q

phakomatoses

A

aka neurocutaneous disorders; lesions in the central and peripheral nervous system, skin, eyes, and other organs

21
Q

most common cause of hypotonia

A

cerebral hypotonia; you know this is cerebral when you see other signs of CNS dysfunction like seizures, developmental delay, etc

22
Q

treatment for ADHD

A

stimulatnes like methylphenidate and dextroamphetamine

23
Q

what is the metabolic defect in Tay Sachs?

A

hexosaminidase A

24
Q

features of tay sachs

A

cherry-red spot

25
metabolic defect in Niemann-Pick disease
sphingomyelinase
26
defect in gaucher disease
glucocerebrosidase; cherry red spot and gaucher cells in bone marrow\
27
defect in Krabbe disease
galacytosylceramide; globoid cells with PAS-pos granules
28
Hurler's syndrome
defect in a-L-iduronidase; clouding of cornea; characteristic facies and dwarfism
29
Hunter's syndrome
defect in iduronate sulfatase; hurler phenotype without clouding
30
metachromatic leukodystrophy
defect in Arylsulfatase A; cherry red psot, demyelinating disorder. Can present as schizophrenia in adults. Pos urine sulfatides
31
adrenoleukodystrophy
defect in very long chain FAO; x linked; white matter hyperintensity on MRI; may present as a neuropahty or myelopathy in adults
32
Alexander disease
defect in glial fibrillary acidic protein; Rosenthal fibers on bx; Macrocephaly; dysmyelination of the CNS
33
Canavan disease
defect in aspartoacylase; macrocephaly, dysmyelination o the CNS
34
Pelizaeus-Merzbacher disease
defect in proteolipid protein; pendular nystagmus; dysmyelination of the CNS
35
Leigh disease
mitochondiral defect; bilateral putaminal hyperintensity on MRI
36
Rett's syndrome
defect in methyl-CpG-binding protein 2; x-linked; occurs exclusively in girls; microcephaly, autism, and hand-wringing
37
neuronal ceroid lipofscinosis
excess lipofuscin storage; dementia, myoclonus, ataxia, retinitis pigmentosa
38
NF1
optic nerve gliomas; caf au lait spots, neurofibromas, axillary or inguinal freckles; Lisch nodules in the iris
39
NF2
bilateral acoustic gliomas
40
tuberous scerosis
cortical tubers, subependymal nodules and astrocytomas, mental retardation, seizures; adenoma sebaceum, ash-leaf spots, shagreen patches; angiomyolipomas of kidneys, cardiac rhabdomyoma
41
ataxia telangiectasia
truncal ataxia, progressive dementai; teleangiectasias; immunodef and susceptibility to infections, leukemia, lymphoma
42
von Hippel-Lindau
cerebellar hemangioblastomas, ataxia; caf au lait spots; renal lesions inluding hemangiomas and carcinomas, pheochromocytoma
43
Sturge weber syndrome
venous angioma of the pia mater, seizures, hemiparesis, mental retard; port-wine stain in the distrib of the ophthalmic nerve