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ABPP > Brain development & childhood disorders > Flashcards

Brain development & childhood disorders Flashcards

1
Q

Fetal alcohol syndrome diagnosis requires abnormalities in what 3 domains?

A

Growth deficiency, specific pattern of facial anomalies, evidence of CNS dysfx

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1
Q

Klinefelter syndrome

A

Extra X chromosome XXY VIQ

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1
Q

Neimann-Pick disease Type C

A

Seizures, gradual onset of dementia, motor abnormalities, mortality by age 40; includes diffuse atrophy with NFTs throughout cerebral cortex & cerebellum

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2
Q

From what germ cell layer do the meninges arise?

A

Mesoderm

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2
Q

Neuropsychological outcomes of spina bifida

A

Motor speech deficits, lower than expected overall cognitive ability, nonverbal < verbal, deficits in reading & listening comprehension likely due to difficulties in making inferences, explicit memory deficits, difficulty w/ attentional control, higher rate of behavioral problems, problems with self-esteem, & adaptive bx

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2
Q

If perinatal stroke is large, how does the brain compensate?

A

Rely on small portion of corticospinal tract that does not cross (uninjured hemisphere to paretic limb)

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2
Q

Periventricular leukomalacia

A

Injury in the periventricular white matter; can affect migration of fibers to the cortex

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3
Q

If perinatal stroke is small, how does the brain compensate?

A

Show intact crossing of corticospinal connections from injured hemisphere to paretic limb

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4
Q

Primary processes responsible for brain damage in VLBW/VPTB

A

hypoxic ischemia w/ associated deprivation of oxygen & glucose, exposure to fetal & maternal infection

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5
Q

Full-term perinatal stroke is most commonly seen in the distribution of what artery? Pre-term perinatal stroke is more often associated with?

A

MCA; periventricular hemorrhagic infarctions

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6
Q

Migration

A

Neuroblasts move to permanent location via radial glia; problems can lead to neurons in the wrong layer of the brain, abnormal connections

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7
Q

Ataxia-telangiectasia

A

Abnormality on chromosome 11 that interferes with DNA repair Progressive ataxic gait due to degeneration of cerebellar vermis; develop telangiectasis of the conjunctiva , chronic sinus & respiratory infections

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8
Q

Patau syndrome

A

Trisomy 13; Microcephaly & sloping head, lowest ears, cleft lip & palate, impaired hearing, severe MR, death in early childhood

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9
Q

Neurobehavioral outcome in patients with VLBW/VPTB

A

Deficits in motor/perceptual motor, exec dysfx, problems in math & sometimes reading, attention, memory

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9
Q

Effects of lead poisoning in children (blood lead concentrations exceeding 60-80 pg/dL)

A

Early symptoms - abdominal pain & arthralgia Progressive encephalopathy characterized by lethargy, anorexia, irritability, ataxia, loss of mental developmental milestones Doss-related declines in IQ, particular effects on attention/exec fx & visuospatial skills

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10
Q

Effects of prenatal maternal stress on cognition & behavior

A

LBW, hyperactive, irritable, irregular feeding, sleeping, bowel movements, crying

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11
Q

Angelman syndrome

A

Due to deletion on chromosome 15 inherited from mother Stereotyped involuntary & jerky-ataxic voluntary movements, smiling face, paroxysms of unprovoked laughter Assoc. w/ motor & severe MR, microcephaly, epilepsy

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11
Q

Pruning

A

Elimination of neuronal overproduction

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12
Q

Megalencephaly

A

Large head circumference (>2 SDs above the mean); involves increased #s of neurons & glial Typically MR, but may be mild

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13
Q

Common brain abnormalities associated with VLBW/VPTB

A

hemorrhagic infarctions associated with germinal matrix IVH, ventriculomegaly, periventricular leukomalacia

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14
Q

3 stages of Piaget’s constructivism

A

1) Sensorimotor (0-2): object permanence, causality, symbolic/representational thought 2) Preoperational (2-7): limited by magical thinking, animism, egocentrism, lack of conservation 3) Concrete Operational (7-11): learn conservation, reflective self 4) Formal Operational (11+): abstraction, relativism, hypothetical-deductive reasoning

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14
Q

Polymicrogyria

A

Development of many small gyri; associated with LD, severe MR, epilepsy

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15
Q

XYY syndrome

A

Tendency toward higher activity levels, more negative mood, temper tantrums Delayed speech acquisition & other neurodevelopmental milestones, psych difficulties, avg-below normal IQ

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16
Q

Describe the pattern of CNS development (i.e., direction)

A

Head -> tail (cephalic -> caudal); near -> far (proximal -> distal), inferior -> dorsal (subcortical -> cortical)

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17
Q

Agenesis of the corpus callosum is associated with an increased incidence of what psychopathology?

A

Schizophrenia

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18
Q

What are some influences on prenatal brain development?

A

Physiology of egg & sperm, intrauterine environment, genetic transmission, errors, mutations, mother’s stress hormones & self-care behaviors

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19
Q

Sturge-Weber syndrome

A

Facial (port wine stain in region of CN V) & cerebrovascular malformations Tend to have MR, LD, behavioral disturbances, refractory epilepsy, may also have focal deficits depending on sit of lesion

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19
Q

Hurler syndrome (MPSI)

A

Autosomal recessive disease with genetic abnormality on chromosome 4, results in glycosaminoglycans (GAGs) not being degraded properly by lysosomes

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20
Q

Neurobehavioral consequences of prenatal exposure to rubella

A

Classic triad: heart disease, vision problems, hearing loss

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20
Q

Cranium bifidum & encephalocele

A

Skin-covered brain, meninges, or CSF protrudes through the skull defect

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21
Q

Spina bifida affects the development of the brain in what 2 major ways?

A

1) failure of neuroembryogenesis results in anomalies in the regional development of the brain 2) children w/ myeloceningocele experience additional injury to the brain b/c of hydrocephalus & its treatment

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21
Q

How is language development affected by perinatal stroke?

A

Order of emergence of skills remains the same but demonstrate delays at each new level of linguistic challenge regardless of which hemisphere is involved In RH stroke, LH remains dominant; B representations emerge in LH stroke

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21
Q

Prader Willi syndrome

A

Deletion on chromosome 15 inherited from father Obese, hyperphagia, poorly controlled food-seeking behavior, MR, OC personality, major language production deficits, hypogonadism, outbursts of aggressive behavior

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22
Q

2 types of periventricular leukomalacia

A

Cystic (lesions around ventricles), noncystic (WM injury)

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24
Q

What portion of children with myelomeningocele are missing one or more parts of the corpus callosum?

A

about 1/2

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24
Q

Untreated PKU leads to

A

Severe MR, decreased attention, lack of responsiveness to environmental stimuli, seizures, spasticity, hyperactive reflexes, tremors, psych illness

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25
Q

What are the 4 major concurrent aspects of cytodifferentiation?

A

1) Development of cell body 2) Selective cell death (40-75% of all neurons die during development) 3) Axonal & dendritic development 4) Synaptogenesis

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26
Q

Williams syndrome

A

Disruption of elastic properties of arteries, root of aorta, skin, & other organs No gross neuro abnormalities but motor delays & fine & gross motor clumsiness; mild-mod MR; imparied reading & writing, visuospatial deficits, difficulty w/ nonverbal tests

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26
Q

Duchenne muscular dystrophy

A

Absence of dystrophin in muscle; associated with mild MR

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27
Q

White matter abnormalities associated with perinatal stroke

A

Decreased CC size related to sections that would have connected the affected site to the other hemisphere, decreased white matter volume in uninjured hemisphere

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28
Q

Which part of the brain is last to be myelinated?

A

Prefrontal areas

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30
Q

Cerebellum development follows a ______ pattern

A

Outside-in

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31
Q

In what ways can treatment for childhood cancer effect the CNS?

A

Cortical atrophy, leukoencephalopathy, mineralizing microangiopathy

32
Q

When do neural tube defects occur?

A

3-4 weeks gestation

33
Q

Risk factors for childhood psychopathology

A

Severe marital discord, low SES, overcrowding, parent criminality, maternal psychopathology, child placed outside the home

34
Q

Dandy-Walker malformation

A

Posterior portion of the upper neural tube fails to develop, child is born with rudimentary posterior brain structures; frequent severe mental retardation

35
Q

What maternal infections have significant effects on prenatal development?

A

Syphilis, toxoplasmosis, rubells, cytomegalovirus, herpes simplex, mumps, hepatitis, chicken pox

36
Q

Lissencephaly

A

Agyria or pachygyria caused by arrest of migration of neuroblasts from periventricular matrix to cortex Assoc. w/ severe growth & developmental retardation, decreased spontaneous activity, difficulty swallowing or eating, minimal response to visual or auditory stimuli

37
Q

Tay Sachs disease

A

Lysosomal storage disorder Primarily affects Ashkenazi Jews

37
Q

Neimann-Pick disease Type B

A

Visceral form, little to no neuro involvement, survival into teens or early adulthood

38
Q

NP deficits associated with childhood cancer treatment

A

10 pt drop in IQ; nonverbal < verbal; arithmetic < reading; nonverbal memory < verbal memory, attention problems, difficulty focusing & planning responses, exec deficits, diminished response time & motor speed

39
Q

Even when treated, patients with PKU may show

A

Exec fx deficits, ADHD symptoms, < avg IQ

40
Q

Spina bifida occulta

A

Occurs when vertebral arches fail to close, resulting in an opening in the spine but no other abnormalities in spinal cord or sac; may be present in at least 5% of the general population

42
Q

Which subtype of cerebral palsy is most closely related to prematurity?

A

Spastic paresis

43
Q

What is the age of peak incidence of acute lymphoblastic leukemia

A

3-5 years

44
Q

Neurofibromatosis Type II

A

B acoustic neuromas that impair hearing until deaf

45
Q

Bronfenbrenner’s ecological model

A

Microsystem (family, school) Mesosystem (microsystems interacting) Macrosystem (environment/society) Exosystem (environmental influences on microsystem)

47
Q

Hallmark feature of fetal alcohol exposure

A

Attention deficit; also see exec fx deficits throughout lifespan

48
Q

Porencephaly

A

Large cystic lesion develops due to disturbed cortical development between months 5-7 gestation

49
Q

Cognitive profile of Down’s syndrome

A

Mean IQ ~ 44.3 Visuospatial abilities generally preserved & language impaired, motor delays, intact social skills, poor memory

50
Q

When to brain divisions occur in the embryo?

A

5-7 weeks

52
Q

Acute disseminated encephalomyelitis

A

Acute demyelinating disease that typically follows infection

53
Q

Critical periods

A

A window of opportunity during which skills need to be consolidated so that that particular system can then establish interconnections with other systems

54
Q

Aggregation

A

Cellular masses or layers are formed by lamination, w/ neurons adhesing together or aligning with neighbors

55
Q

Tuberous sclerosis

A

Sclerotic masses (scarring due to glial overgrowth) form in cerebral cortex, usually resulting in MR & epilepsy; also results in adenoma sebaceum (nodules on face)

57
Q

Resiliency factors against childhood psychopathology

A

Reduced stress, easy temperament, stable support

59
Q

Cerebral hemisphere development follows a ______ pattern

A

inside-out

61
Q

What complications/disabilities are associated with cerebral palsy?

A

MR, cognitive dysfx, epilepsy, vision/hearing impairments, LD, language disorders, psych problems, pseudobulbar palsy, hyperactivity

62
Q

Lesch-Nyhan disorder

A

Inborn error of purine meatbolism leading to increased levels of uric acid in blood & urine MR in most, movement disorder including choreoathetosis & spasticity, self mutilation

64
Q

Stages of prenatal brain development

A

Proliferation, migration, aggregation, cytodifferentiation, pruning

65
Q

Cerebral palsy

A

Permanent, nonprogressive neurologic motor system impairment resulting from CNS injuries of the immature brain

66
Q

3 main processes for CNS elaboration

A

Dendritic arborization, myelination, synaptogenesis

67
Q

Presentation of cri-du-chat syndrome

A

Abnormal cry like a kitten, severe MR, epicanthal folds, moon face

69
Q

Which subtype of cerebral palsy is less likely to be associated with epilepsy or MR?

A

Extrapyramidal

70
Q

Teratogens

A

Substances that can cross the placenta and lead to birth defects

72
Q

Meningocele

A

Malformation in which the meninges alone herniate through the spinal defect, creating a sac that is filled with cerebral spinal fluid; can affect ambulation, bladder/bowel control

73
Q

Neurobehavioral consequences of prenatal cocaine exposure

A

Small for gestational age, seizures, LBW, exaggerated startle, increased tone & motor activity, impaired fine motor coordination, auditory impairments, high-pitched cry, sleep & feeding problems, difficulty to comfort, developmental delays, bx & attentional problems

74
Q

Acute lymphoblastic leukemia

A

Cancer of blood-forming cells in bone marrow

76
Q

Two subtypes of cerebral palsy

A

Spastic paresis (most common), extrapyramidal

77
Q

Proliferation

A

Cell generation by mitosis inside the neural tube; problems can lead to fewer neurons at the beginning of life (e.g, MR)

78
Q

Global cognitive & academic deficits associated with VLBW/VPTB

A

Low average range, higher rates of grade retention & sped, internalizing & externalizing behavior, smaller than peers

80
Q

Neurofibromatosis Type I

A

Triad of cafe au lait spots, neurofibromas, & Lisch nodules Assoc. w/ LD & ADHD, MR-avg IQ, deficits in language, reading, spelling, exec fx, visuospatial, visuomotor skills

81
Q

Effects of low-level prenatal lead exposure

A

Mild growth retardation in utero & mild developmental delay up to ages 1-2, recovery by school age

82
Q

What are the three main processes for CNS elaboration?

A

Dendritic arborization, myelination, synaptogenesis

83
Q

Turner’s syndrome

A

XO; no consistent NP profile, but at risk for NVLD; small stature, poorly developed secondary sex characteristics, sexual dysfx in puberty/adulthood, webbed neck, broad chest

84
Q

Edwards syndrome

A

Trisomy 18; slow growth, seizures, severe MR, lowest ears, mottled skin, clenched fist with 3rd finger over index finger, death in early infancy

86
Q

How is visual pattern processing affected by perinatal stroke?

A

Do not show progression from piecemeal to global-local integration at 10-12 years old RH stroke = retention of details/local characteristics but no global characteristics; LH stroke = retention of global characteristics w/ paucity of details

87
Q

CNS abnormalities seen in Turner’s syndrome

A

Increased ventricular volume, increased CSF, decreased gray matter & white matter in parietal/occipital lobe

88
Q

Risk factors associated with childhood cancer treatment effects

A

Age (

89
Q

Leukodystrophies

A

Inherited deficiency of a specific enzyme; abnormalities interfere with enzyme or protein synthesis; results in buildup of neurotoxins in the brain

91
Q

Clinical presentation of acute disseminated encephalomyelitis

A

Confusion, somnolence, seizures, fever, stiffness of neck, ataxia, myoclonus, choreoathetosis

92
Q

Heterotopias

A

Focal abnormalities of cortical cytoarchitecture; areas of disordered layering & displaced cells Reported in patients with epilepsy & LD

93
Q

Which syndrome is often associated with extraordinary talents in music & verbal fluency?

A

Williams syndrome

94
Q

Chiari II malformation

A

Small cerebellum & brainstem, herniation of cerebellum & portions of medulla & pons through an enlarged foramen magnum

95
Q

Risk factors for negative outcome in VLBW/VPTB

A

Lower birthweight, younger gestational age, neonatal neurological complications of IVH & PVL, chronic lung disease, environmental disadvantages

95
Q

Hydranencephaly

A

Cerebral hemispheres replaced by cystic sacs containing CSF

96
Q

Environmental influences on development of spina bifida

A

Folic acid deficiency, maternal diabetes & obesity, maternal illness & hyperthermia

97
Q

Neimann-Pick disease

A

Group of inherited disorders causing disruption in individual’s ability to metabolize cholesterol & other lipids

99
Q

Rett’s syndrome

A

After 6 months of normal development, regression in all areas of psychomotor development over several years leading to profound MR, acquired microcephaly Abnormality is assumed lethal in males

100
Q

What type of glia are involved in migration?

A

Radial glia & Bergmann glia

101
Q

Neimann-Pick disease Type A

A

Severe neurological & physical deficits, death by age 4

102
Q

Cytodifferentiation

A

Development of cells into different functions, formation of axonal & dendritic processes

103
Q

Neurobehavioral consequences of prenatal narcotic exposure

A

Neonatal abstinence syndrome, LBW, small head, SIDS, childhood academic problems

104
Q

Females with Fragile X syndrome present with

A

Relative strengths in verbal & facial recognition, relative weaknesses in visuospatial processing & exec fx

105
Q

Myelomeningocele

A

Involves the protrusion of spinal cord along with the meninges through the spinal defect; more likely to be associated with severe brain abnormalities or major neurobehavioral deficits

106
Q

Neuropsychological deficits associated with acute disseminated encephalomyelitis

A

Deficits in attention/concentration, memory, language, visuoperception, executive, and/or motor skills Can see global impairment after recovery with severe ADEM, assoc. w/ the extent of underlying neuropathology present

107
Q

Effects of maternal cytomegalovirus on fetus

A

Enlargement of spleen & liver, intrauterine growth retardation, various congenital malformations, damage to visual & auditory systems, microcephaly, MR

ABPP (34 decks)

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