Cerebral Palsy 249.
e2
• Spasticity WORKUP
BASIC INFORMATION • Athetosis • Laboratory tests are not necessary to establish
• Delay in motor milestones the diagnosis.
DEFINITION • Hyperreflexia • Workup is helpful for assessment of recur-
Cerebral palsy (CP) is a group of disorders of the • Seizures rence risk, implementation of prevention pro-
central nervous system characterized by aberrant • Developmental delay in many individuals grams, and medicolegal purposes.
control of either movement or posture, present • Mental health disorders: Compared with
since early in life and not the result of a progres- adults without CP, those with CP have an LABORATORY TESTS
sive or degenerative disease. Table E1 provides a elevated prevalence of mental health disorders, • Metabolic and genetic testing should be consid-
classification of CP and major causes. CP can be some of which may be more pronounced in ered if on follow-up the child has the following:
characterized further by the affected parts of the patients with comorbid neurodevelopmental (1) Evidence of deterioration or episodes of
body (Table E2) and descriptions of the predomi- disorders1 metabolic decompensation, (2) no etiology
nant type of motor disorder (Table E3). determined by neuroimaging, (3) family his-
ETIOLOGY tory of childhood neurologic disorder associ-
SYNONYMS Multifactorial, including low birth weight, con- ated with CP, or (4) developmental malforma-
CP genital malformation, asphyxia, multiple gesta- tion on neuroimaging. Hypercoagulability
Little disease tion, intrauterine exposure to infection, neonatal
Congenital static encephalopathy stroke, hyperbilirubinemia, and maternal thyroid
Congenital spastic paralysis malfunction. Risk factors for cerebral palsy are TABLE E2 Descriptions of
summarized in Table E4. Cerebral Palsy by Site of
ICD-10CM CODES
Involvement
G80.0
G80.1
Spastic quadriplegic cerebral palsy
Spastic diplegic cerebral palsy
DIAGNOSIS
Hemiparesis (hemiplegia): Predominantly unilateral
G80.2 Spastic hemiplegic cerebral palsy A motor deficit is always present. The usual impairment of the arm and leg on the same (e.g.,
G80.3 Athetoid cerebral palsy presenting complaint is that child is not reach- right or left) side
G80.4 Ataxic cerebral palsy ing motor milestones at the appropriate age. Diplegia: Motor impairment primarily of the legs (of-
G80.8 Other cerebral palsy Medical history establishes that the child is not ten with some limited involvement of the arms;
G80.9 Cerebral palsy, unspecified losing function (i.e., that deficit is static and not some authors challenge this specific type as not
progressive). This history, combined with a neu- being different from quadriplegia)
rologic examination establishing that motor Quadriplegia: All four limbs (whole body) are func-
EPIDEMIOLOGY & deficit is due to a cerebral abnormality, estab- tionally compromised.
DEMOGRAPHICS lishes the diagnosis of CP. Serial examinations
INCIDENCE (IN U.S.): 2 to 2.5 persons per 1000 From Marcdante KJ et al: Nelson essentials of pediatrics,
may be necessary. ed 9, Philadelphia, 2023, Elsevier.
live births
PREDOMINANT SEX: Males and females af- DIFFERENTIAL DIAGNOSIS
fected equally Other causes of neonatal hypotonia include TABLE E3 Classification of
PREDOMINANT AGE: Diagnosis typically made muscular dystrophies, spinal muscular atrophy, Cerebral Palsy by Type of Motor
at 3 to 5 yr of age Down syndrome, and spinal cord injuries. Disorder
Causes of progressive spasticity in children in-
PHYSICAL FINDINGS & CLINICAL clude metabolic diseases involving white matter Spastic cerebral palsy: The most common form of
PRESENTATION (e.g., leukodystrophy). Slowly progressive disor- cerebral palsy, it accounts for 70%-80% of cases.
• Monoplegia, diplegia, quadriplegia, hemiplegia It results from injury to the upper motor neurons
ders sometimes misdiagnosed as cerebral palsy
of the pyramidal tract. It may occasionally be
• Often hypotonic in newborn period, followed are summarized in Table E5. bilateral. It is characterized by at least two of the
by development of hypertonia following: Abnormal movement pattern, increased
tone, or pathologic reflexes (e.g., Babinski
TABLE E1 Classification of Cerebral Palsy and Major Causes response, hyperreflexia).
Dyskinetic cerebral palsy: Occurs in 10%-15% of
Motor Syndrome cases. It is dominated by abnormal patterns of
(Approx. % of CP) Neuropathology/MRI Major Causes movement and involuntary, uncontrolled, recurring
Spastic diplegia (35%) Periventricular leukomalacia Prematurity movements.
Periventricular cysts or scars in white Ischemia Ataxic cerebral palsy: Accounts for ,5% of cases.
matter, enlargement of ventricles, Infection This form results from cerebellar injury and fea-
squared-off posterior ventricles Endocrine/metabolic (e.g., thyroid) tures abnormal posture or movement and loss of
Spastic quadriplegia (20%) Periventricular leukomalacia Ischemia, infection orderly muscle coordination or both.
Multicystic encephalomalacia Endocrine/metabolic, genetic/ Dystonic cerebral palsy: Also uncommon. It is char-
Cortical malformations developmental acterized by reduced activity and stiff movement
Hemiplegia (25%) Stroke: Thrombophilic disorders (hypokinesia) and hypotonia.
In utero or neonatal Infection Choreoathetotic cerebral palsy: Rare now that
Focal infarct or cortical, subcortical excessive hyperbilirubinemia is aggressively
Genetic/developmental prevented and treated. This form is dominated by
damage
Cortical malformations Periventricular hemorrhagic infarction increased and stormy movements (hyperkinesia)
Extrapyramidal Asphyxia: Asphyxia and hypotonia.
(athetoid, dyskinetic) Symmetric scars in putamen and thalamus Kernicterus Mixed cerebral palsy: Accounts for 10%-15% of
(15%) Kernicterus: cases. This term is used when more than one
Mitochondrial type of motor pattern is present and when one
Scars in globus pallidus, hippocampus
Mitochondrial: Genetic/metabolic pattern does not clearly dominate another. It
Scarring of globus pallidus, caudate, typically is associated with more complications,
putamen, brainstem including sensory deficits, seizures, and cognitive-
No lesions: ? dopa-responsive dystonia perceptual impairments.
CP, Cerebral palsy; MRI, magnetic resonance imaging. From Marcdante KJ et al: Nelson essentials of pediatrics,
From Kliegman RM: Nelson textbook of pediatrics, ed 21, Philadelphia, 2020, Elsevier. ed 9, Philadelphia, 2023, Elsevier.
Descargado para Dra Mariela Bautista Zamata (bautistazamatamariela1@gmail.com) en Pontifical Xavierian University de ClinicalKey.es por Elsevier en junio
16, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados.
249.e3 Cerebral Palsy
TABLE E5 Slowly Progressive ACUTE GENERAL Rx
TABLE E4 Risk Factors for
Cerebral Palsy Disorders Sometimes If present, treatment of seizures
Misdiagnosed as Cerebral Palsy
Pregnancy and Birth CHRONIC Rx
Low socioeconomic status Condition • Treatment of seizures, as directed by seizure
Prematurity Polyneuropathy type.
Low birthweight/fetal growth retardation (,1500 g GM1 gangliosidosis type II • Medical treatment of spasticity includes
at birth) Hereditary motor and sensory neuropathies baclofen (oral and intrathecal), as well as
Maternal seizures/seizure disorder Infantile neuroaxonal dystrophy botulinum toxin A.
Maternal treatment with thyroid hormone, estrogen, • Surgical treatments of spasticity include
Metachromatic leukodystrophy
or progesterone dorsal rhizotomy, tendon lengthening, and
Ataxia
Pregnancy complications osteotomy.
Abetalipoproteinemia
Polyhydramnios
Eclampsia Ataxia-telangiectasia DISPOSITION
Third-trimester bleeding (including threatened abortion Friedreich ataxia Most children with CP live at home. Those
and placenta previa) Spasticity-chorea children with severely impaired mobility or
Multiple births Familial spastic paraplegia other disabilities may live in chronic-care nursing
Abnormal fetal presentation Glutaric aciduria type I facilities.
Maternal fever Lesch-Nyhan syndrome
Congenital malformations/syndromes REFERRAL
Niemann-Pick disease type C
Newborn hypoxic-ischemic encephalopathy If the child has difficulty with spasticity, physical
Pelizaeus-Merzbacher disease
Bilirubin (kernicterus) medicine and rehabilitation referrals are espe-
Rett syndrome
Acquired After the Newborn Period cially helpful.
Meningitis From Pina-Garza J, James KC: Fenichel’s clinical pediatric
Head injury neurology, ed 8, Philadelphia, 2019, Elsevier.
PEARLS &
Car crashes
Child abuse CONSIDERATIONS
Near-drowning IMAGING STUDIES • In full-term infants, there is usually no history
Stroke
• Neuroimaging is recommended if the etiology of traumatic delivery.
From Marcdante KJ et al: Nelson essentials of pediatrics, has not been established previously, for ex- • Compared with delivery at 40 wk gestation,
ed 9, Philadelphia, 2023, Elsevier. ample, by perinatal imaging. delivery at 37 or 38 wk or at 42 wk or later
• MRI, when available, is preferred to com- was associated with an increased risk of CP.
puted tomography scanning because of
higher yield in determining an etiology and RELATED CONTENT
workup should be considered if neuroimag- timing of the insult leading to CP. Cerebral Palsy (Patient Information)
ing is suggestive of a remote stroke.
• Electroencephalogram should be obtained
when a child with CP has a history suggestive TREATMENT AUTHORS: NAOMI R. KASS, BA and
of seizures. JOSEPH S. KASS, MD, JD, FAAN
• Children with CP should be screened for NONPHARMACOLOGIC THERAPY
ophthalmologic and hearing impairments as • Physical therapy, occupational therapy, and
well as speech and language disorders. Nu- speech therapy.
trition, growth, and swallowing function • Orthotics and casting are used to increase
should be monitored. musculotendinous length.
REFERENCE
1. Whitney DG et al: Prevalence of mental health disorders among adults with
cerebral palsy: a cross-sectional analysis, Ann Intern Med 171(5):328-333,
2019.
Descargado para Dra Mariela Bautista Zamata (bautistazamatamariela1@gmail.com) en Pontifical Xavierian University de ClinicalKey.es por Elsevier en junio
16, 2024. Para uso personal exclusivamente. No se permiten otros usos sin autorización. Copyright ©2024. Elsevier Inc. Todos los derechos reservados.
