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51 Cards in this Set
- Front
- Back
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What is cerebral palsy
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Neurodevelopmental impairment cuased by a nonprogressive defect or lesion in single or multiple locations in the immature brain
Can occur in utero, during birth or shortly after birth Produces voluntary motor disturbances and possible sensory impairments Can appear progressive as the child grows and develops -Secondary impairments develop -CP affects the changing organism -Demands placed on child increase as growth occurs |
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Other sx of cerebral palsy
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mental retardation (50-75%)
Speech disorders (25-65%) Hearing impairments (25%) Seizure (25-35%) Abnormal vision (40-50%) Social and family problems |
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Epidemiology of CP
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Very common, second most in childhood after mental retardation
Spike in 50s, drop, now back to 2.5/1000 ^because low birth weight survival improvement |
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Risk factors for CP
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Low birth weight (40x weights less than 1000 grams)
Preterm infants born at 24-28 weeks -50% have no impairments -25% have major impairments -11% have Cerebral Palsy |
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What causes CP?
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Prenatal, Perinatal, Postnatal
Any conditions causing cerebral anoxia 33% of all cases in preterm 50% of spastic diplegia are preterm 5% of quadriplegia are preterm *Spastic diplegia most common form of CP |
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PRENATAL CAUSES of CP
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PRENATAL CAUSES
Nutrition, Dev problems in fetus, Maternal infection (CMV, HIV) |
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Perinatal Causes of CP
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Intracranial Hemorrhage
Neonatal asphyxia (most common birth-28 days) Perinatal infection Hyperbiliruinemia (athetoid) Rh incompatibility b/t mom/baby *Developed countries have higher b/c of infant survival rate |
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Classification of CP
By body area involved |
Monoplegia (rare) - one limb
Diplegia (41.5%) - trunk and LE Hemiplegia (36.4%) - UE/LE on one side, UE more Quadriplegia - all limbs, head and trunk |
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Classification of CP
By most obvious impairment |
Spastic - Most common, 75%
Dyskinetic - 10-20 % -Athetosis - poorly executed in timing, direction and spatial -rigidity - not velocity dependent -tremor - Very rare *BG, prefrontal, premotor Ataxia - 5-10% -disorder of balance, alignment -Oral/motor - dysarthria *Cerebelum Hypotonia Mixed |
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Classification of CP
By severity |
Mild
-Sensorimotor impairments only limiting in advanced gross motor skills -No DME needed but maybe extra time Moderate -functional limitations in whole body fxn (walking, hands, speech) -Modifications to perform task Severe -unable to fulfill normal life roles -QOL maybe serious affected Profound -No useful or purposeful motor ability, even basic functions are absent -aids in all daily activities |
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Types of lesions in CP
Perventricular atrophy (Perventricular leukomalacia) |
Most common abnormality in preterm infants
Reduction of blood flow in vulnerable periventricular region -end zone between ACA, MCA, PCA Uncertain if lesions occur before/during/after birth Bilateral cysts >3mm in diameter in parietal/occipital area - 90% develop CP Lesion usually bilat - spastic diplegia Can be asymmetrical - hemiplegia Can extend outward - quad Damage to optic radiations - visual deficits |
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Types of lesions in CP
Periventricular Intraventricular Hemorrhage (Subependymal Hemmorhage) |
Typically in preterm (<28 wk) and low birth weight
40% in preterms <35 weeks IVH 46% in infants <1500 grams Usually occur in first two postnatal days (most w/i one week) Second leading cause of death after Respiratory Distress Syndrome (RDS) |
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Pathology of Periventricular intraventricular hemorrhages (Subependymal hemorrhages)
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Bleeding into the subependymal matrix below the ventricles
Gelatinous area containing large vascular supply (ACA) Primitive vessels with single layer of epithelium (no smooth m, collogen) Hemorrhage precipitated by perturbations in arterial pressure and flow Blood ruptures into lateral ventricles |
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Neurologic complications of IVH
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Hydrocephalus
Germinal matrix destruction Cyst formation Anoxic encephalopathy |
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Grading of IVH
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I - Isolated Germinal matrix hemorrhage (15% have problems)
II - IVH w/normal size ventricles. Occurs when ruptures into lateral ventricles (30% have problems) III - IVH acute ventricle dilation (40% have problems) IV - hemorrhage into periventricular white matter (90% have problems, spastic di/quad, vision, cognitive) |
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Treatment for IVH
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Support cerebral perfusion by maintaining arterial BP
Avoid cerebral hemodynamic disturbances Serial imaging of ventricles Ventriculostomy and shunting Prophylactic meds (Phenobarbital/seizures, Ethamsylate/reduce bleeding) |
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Types of lesions in CP
Hyperbilirubinemia (physiologic jaundice) |
Premature infants with immature hepatic and increased hemolysis of RBC
Kernicterus - deposition of unconjugated bilirubin in the brain (BG/hippocampus) Treatment Phototherapy, Exchange transfusions |
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Types of lesions in CP
Hypoxic-Ischemic Encephalopathy (HIE) |
Occurs in full term who have experienced perinatal asphyxia
prolonged asphyxia->cytotoxic edema/impaired blood flow->cerebral necrosis Seizures, altered consc., abnormal tone/posture/reflexes/autonomic |
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Causes of Hypoxic-Ischemic Encephalopathy (HIE)
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Altered plpacental exchange, reduced maternal blood flow to placenta, placenta previa, postmaturity, prolapsed umbilical cord, umbilical cord around neck
Intrauterine growth retardation, placental insufficiency, maternal hypotension |
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Neurologic sequelae in Hypoxic-Ischemic Encephalopathy (HIE)
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Normal at one week
Abnormal after 3 weeks - at risk for motor deficits, seizure |
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Classifications of HIE
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Selective Neuronal Necrosis
-Neuronal death widespread -Stupor, coma, seizure, hypotone, Oculomotor and oral problems Status Marmoratus Parasagittal cerebral injury Focal Ischemic brain necrosis |
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Types of lesions in CP
CNS malformations |
CNS malformations may generate hemorrhagic or anoxic lesions
Causes Prenatal drugs, radiation, virus (herpes simplex, rubella, CMV) |
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Correlation between lesion and presentation...
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Correlation between lesion and presentation is impossible
Continuum between hemi and di resulting from perivent lesions Quad often from BS damage in addition to cortical/subcortical |
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Genetic Defects causing metnal retardation
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Many causes in children
-50% unknown (mild) -25% unknown (severe) |
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Cognitive delays associated with CNS pathology
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CP - variable sx and progression
Spina Bifida - cognitive variable, associated with hydrocephalus, shunt placement/infection Also: Anencephaly, Holoprosencephaly, Hydrocephalus, Microcephaly, Agenesis of Corpus Callosum |
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Disorders resulting from prenatal maternal infections or other terratogens
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Cytomegalovirus
Fetal Alcohol syndrome |
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Post natal problems
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Head Injury, Seizure disorders, Infections, Demyelinating disorders
Malnutrition, Environmental deprivation/toxins (Pb poisoning) |
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Cognitive delays associated with metabolic and endocrine problems
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Leukodystrophies
Hurler syndrome Phenylketonuria (PKU) |
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Genetic abnormalities
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Chromosomal
-Trisomy 21 -Trisomy 18 -Trisomy 13 Sex chromosome -Turner syndrome -Klinefelder syndrome Partial deletion -Cri-du-Chat syndrome -Prader-Willi syndrome |
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Genetic abnormalities, continued
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Specific Gene defects
-Osteogenesis imperfecta -Tuberous Sclerosis -Neurofibromatosis Autosomal recessive -Hurler syndrome -Phenylketonuria (PKU) Other -Rett Syndrome -Fragile X syndrome |
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Downs syndrome
Epidemiology |
Most common chromosomal cause of mod/severe mental retardation
1/800 live births Incidence up with maternal age (maybe paternal too) -up to mid 30s 1/100 -45 yo mom 1/30 |
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Downs syndrome
Genetics |
91% have extra 21st chromosome
9% have mosaic and translocation forms |
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Downs syndrome
Pathology |
Small brain 76% of normal
-cerebellum/BS 66% of normal\ -brain narrow AP, wide lat Cytological differences -small amount of small neurons -reduced synaptogenesis -abnormal dentritic spines of pyramidal neurons -decreased myellination of precentral, frontal, cerebellum Alzheimers sx by age 35 -plaques, tangles, loss of hippocampus neurons |
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Downs
newborn characteristics |
Hypotonicity, Poor Moro
Joint hyperextensibility Excess skin on back of neck Flat face, large tongue, epicanthal folds, speckled iris Dysplasia of middle phalanx 5th finger Simian creases |
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Downs
Development in infancy/early childhood |
Hypotonicity (98%), low muscle force, persistent primitive reflexes
Poor dev of postural tone, slow automatic postural reactions motor delays increase with age |
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Downs
Associated impairments |
Congenital heart disease (40%)
Lung hypoplasia with pulmonary HTN MSK - Metatarsal varus, pes planus, TL scoliosis, patellar instab, hip sublux, AA instab feeding problems - shortened palate, oral hypotonia, tongue thrusting, poor lip closure Sensory impairments - astigmatism, strabismus, nystagmus, hearing loss MR - progressive IQ loss\ small stature, leukemia risk 15x |
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Trisomy 18
Edwards syndrom |
1/8000 birth, F>M, advanced maternal age
Only 10% survive >1 year malformed CV, GI, urogenital, skeletal Microcephaly, cerebellar, myelomenigocele Profound MR, feeding problems |
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Trisomy 13
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1/15-20k, advanced maternal age, only 10% survive >1year
Microcephaly, cleft lip and palate Profound MR, other CNS malformations |
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Turner Syndrome
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Sex chromosome
1/2500, not related materal age Sexual infantilism, congenital webbed neck, cubitus valgus Growth retardation, lack of sexual dev, sterile congenital heart, kidney problems Skeletal abnormalities (hip dislocation, pes planus, dislocated patella) Incidence of MR>general pop. |
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Klinefelter Syndrom
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XXY
1/1000 males. Not evident till puberty XXY - normal intelligence, passive XXXY/XXXXY - severe MR, multiple anomalies |
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Cri du Chat
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Partial deletion of short arm chromosome 5
1-20/50k, F>M High pitched cry (pharynx dev), microcephaly, hypotonia Strabismus, moon face, low ears MSK - scoliosis, hip dislocate, club feet, hypermobile feet/hands |
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Prader Willi
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Partial deletion, 1/25k
Obesity, hypogonadism, short stature, dysmorphic face Dysfunctional CNS Food obsession Hypotonia - poor infant feeding, causing 2ndary msk problems Mild/mod MR, BEHAVIOR |
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Osteogenesis Imperfecta
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condition causing extremely fragile bones
Autosomal dominant |
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Tuberous Sclerosis
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Autosomal dominant
Seizures, MR, Sebacious adenomas, delayed development |
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Neurofibromatosis
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Autosomal dominant
Light brown skin pathces Neurofibromas in CNS/PNS -CN II & VIII damage -Para, quad, hemiparesis -maybe dev on stomach, kidneys, heart |
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Hurler Syndrome
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Autosomal recessive, 1/100k, inborn error of metabolism
Normal at birth, deteriorate, death before adult |
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SMA spinal muscular atrophy
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Spinal Muscular Atrophy (SMA) is a neuromuscular disease characterized by degeneration of motor neurons,resulting in progressive muscular atrophy (wasting away) and weakness.
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Phenlketonuria
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Inborn error metabolism
Autosomal recessive, progressive deterioration and MR, PREVENTABLE |
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Hemophilia A
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Sex linked disorder
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Fragile X
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Sex linked disorder
1/1250 males, 1/2500 females Fragile site on long arm X Normal lifespan 80% males have MR Delayed motor skills, emotional lability, autistic-like |
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Rett Syndrome
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Sex linked, 1/15k female live births
Normal till 6mo then progressive deterioration Lose language and hand skills Profound MR Delayed/Absent walking Hypotonicity then hypertonicity Seizures |
