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ABCN Exam August 2021 > ABCN Deck 5 > Flashcards

ABCN Deck 5 Flashcards

1
Q

genetic syndrome characterized by elevated phenylaline in blood and low levels of tyrosine; untreated, it can cause significant neurological dysfunction. treated, cognitive/behavioral characteristics can include ADHD, math deficits and reduced processing speed.

A

phenylketonuria

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2
Q

Benzodiazepine used to reduce spasticity, hyperreflexia, and muscle spasms in CP

A

diazepam, valium

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3
Q

NF1 neuropsychological profile typically includes weaknesses in:

A

academics, attention, EF, visuospatial skills, word list generation, naming, social awkwardness; intact memory

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4
Q

__% of people with NF1 have brain tumors

A

15

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5
Q

___ has become the most widely used standardized measure of CP severity in children.

A

Gross Motor Function Classification System

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6
Q

genetic syndrome characterized by adrenocortical insufficiency, nonverbal deficits, EF deficits, psychiatric symptoms in adulthood

A

Adrenoleukodystrophy

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7
Q

___ is the leading cause of disability in childhood.

A

CP

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8
Q

___ is a non-invasive method designed to qualitatively assess spontaneous movements in children born preterm through approximately 20 weeks post-term corrected age.

A

General Movement Assessment (GMA)

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9
Q

genetic syndrome characterized by port wine stain, vascular malformations of the eye and brain, seizures, migraines, stroke-like episodes, ID.

A

Sturge-Weber Syndrome.

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10
Q

Genetic syndrome characterized by connective tissue abnormalities, cardiovascular disease, ocular & auditory abnormalities, elfin appearance, starburst iris, irregular dentition, ID, verbal > nonverbal, hypersociability, anxiety/phobias

A

Williams Syndrome

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11
Q

For CP, ___ independently by age 2 is predictive of eventual ambulation.

A

sitting

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12
Q

For preterm infants, ___% of all IVH occur in the first 6 to 8 hours of life and ___% occur within the first 4 to 5 days.

A

50, 95

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13
Q

__ to __% of people with NF1 have macro- or megalencephaly.

A

30 to 50

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14
Q

__% of children with TSC are diagnosed with ID.

A

45

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15
Q

genetic syndrome characterized by prominent ears, hyperextensive joints, flat feet, soft skin, long face, epilepsy, macroorchidism, autism, ADHD, anxiety/depression

A

Fragile X

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16
Q

genetic disorder characterized by ataxic gait, epilepsy, microcephaly, prominent chin, deep set eyes, wide mouth, protruding tongue, severe ID, speech delays, happy disposition with easily provoked laughter

A

Angelman Syndrome

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17
Q

___ is the 2nd most important risk factor for CP.

A

low birth weight

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18
Q

up to __% of children and __% of adults with CP experience chronic pain.

A

75, 70

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19
Q

80 to 90% of individuals with ___ are diagnosed with epilepsy. seizures typically begin in infancy and are often intractable.

A

TSC

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20
Q

Fetal risk factors for CP Include (6)

A

multiple birth gestation, death in-utero of cotwin, male sex, IGR, brain malformations, thrombolic disorders

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21
Q

genetic syndrome characterized by short stature, webbed neck, cardiovascular malformations, congential heart disease, kidney malformations, hearing loss, strabismus, hypothyroidism, infertility, ADHD, math disability, nonverbal deficits, social cognition deficits, depression in adulthood

A

Turner syndrome

22
Q

__ is the foremost risk factor for CP.

A

prematurity

23
Q

___ is a model of medical management used in NICUs. It attempts to replicate the intrauterine environment.

A

NIDCAP

24
Q

__ to __% of children with NF1 have focal areas of hyperintensity of t2-weighted MRIs.

A

60-70

25
Q

In children with NF1, UBO’s frequently occur in ____ (5)

A

basal ganglia, cerebellum, thalamus, brainstem, subcortical white matter

26
Q

The ___ system connects regions of the cortex involved with motor control to muscle via the corticospinal tract. it is responsible for initating and carryibg signals that enable voluntary skilled movements.

A

pyramidal

27
Q

Diagnostic criteria for NF1 include the presence of two of the following (7):

A

6+ cafe au lait macules, 2+ neurofibromas, freckling in the armpit or groin, optic glioma, 2+ Lisch nodules, distinctive bony lesion, 1st degree relative with NF1

28
Q

_____ is the most commonly used imaging technique in the context of prematurity to detect neurological events and abnormalities.

A

serial cranial ultrasound

29
Q

which CP subtype? extrapyramidal system. hypoxic/ischemic injury with basal ganglia involvement, perinatal injury.

A

dyskinetic.

30
Q

which CP subtype? extrapyramial system. cerebellar malformation.

A

ataxic-hypotonic.

31
Q

For the preterm infant, increased ____ and ____ lead to decreased risk for long-term impairment.

A

gestational age, birth weight

32
Q

a heart condition in which the ductus arteriosus of the heart does not close

A

patent ductus arteriosus

33
Q

Which CP subtype? Pathogenesis in pyramidal motor system. unilateral MCA stroke, prenatal injury.

A

spastic hemiplegia

34
Q

which CP subtype? pathogenesis in pyramidal system. anoxia or other generalized insult/bilateral gray matter injury, prenatal in 50% of cases.

A

spastic quadriplegic.

35
Q

genetic syndrome characterized by tall stature, hypogonadism, fertitlity problems, hypophonia, clinodactyly, hyperextensible joints, flat feet, language deficits, dyslexia, ADHD, anxiety/depression

A

Klinefelter syndrome

36
Q

In children born preterm, differences are found in the following domains on neuropsych testing (9)

A

IQ, attention, processing speed, fluid verbal skills, visuospatial skills, memory, EF, motor dexterity & coordination, internalizing & externalizing behaviors

37
Q

Predictors of functional outcome for preterm infants include (8)

A

severity of medical/neurological complications, use of steroids, access to NICU/specialists, length of NICU stay, SES and other family/environmental factors, Males at greater risk, growth in NICU, brain volume

38
Q

Maternal risk factors for CP include (5):

A

ID, infection or toxic exposure during pregnancy, epilepsy, thyroid disorder, high blood pressure during pregnancy

39
Q

Neuropathology of TSC include (3)

A

cortical tubers, subependymal nodules, subependymal giant-cell astrocytomas (SEGA)

40
Q

Muscle relaxant used to treat spasticity, hyperreflexia, and muscle spasms in CP

A

baclofen/Lioresal

41
Q

the incidence of epilepsy in individuals with CP is __ to __%

A

28-50

42
Q

sleep disorders occur in __ to ___% of individuals with CP.

A

20-25

43
Q

Neuromuscular blocker used to reduce spasticity, improve range of motion

A

botulinum toxin (Botox)

44
Q

Autosomally dominant neurocutaneous disorder affecting multiple organ systems including the skin, heart, kidney, lungs, and brain. ID & autism.

A

Tuberous Sclerosis Complex

45
Q

genetic disorder characterized by hypotonia, hypogonadism, hyperphagia, obesity, almond shaped eyes, narrow temple and nasal bridge, thin upper lip, short stature, small hands and feet, ID, autism

A

Prader-Willi syndrome

46
Q

Signs of possible CP in infancy include (4)

A

persisting infantile reflex patterns, delayed motor milestones, standing well before age 12 months, strong hand preference in first 12-18 mos

47
Q

In children older than 5 months corrected age, ____ combined with neonatal MRI is more than 90% accurate in predicting CP

A

Hammersmith Infant Neurological Examination (HINE)

48
Q

Genetic syndrome characterized by cardiac defects, mild hearing loss, palatal defects, long face, small ears, narrow eyes, borderline IQ, verbal>nonverbal, anxiety, schizophrenia

A

22q11.2 deletion syndrome

49
Q

half of children with mild CP “outgrow” the disorder by age __.

A

7 years.

50
Q

which CP subtype? pathogenesis in pyramidal motor system. white matter damage of prematurity, prenatal injury.

A

spastic diplegic.

51
Q

Comorbidities commonly seen in people with NF1 include

A

LD in reading, math, written exprsesion, ADHD

52
Q

blood vessels in the retina of the eye develop abnormally; can be caused by premature birth or excessive use of oxygen after birth.

A

retinopathy of prematurity

ABCN Exam August 2021 (19 decks)

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