Cerebral Palsy.

  • Cerebral Palsy is group of disorders of the development of movement and posture causing activity limitations that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain.

ETIOLOGY/ PATHOGENESIS

  • Etiology of CP is not well understood, and brain lesions are thought to be associated with prenatal, perinatal, or postnatal events of varying causes up to age 3 years.
  • Risk factors for CP are multifactorial and can include preterm birth, multiple gestation, intrauterine growth restriction, male sex, low Apgar scores, intrauterine infections, maternal thyroid abnormalities, prenatal strokes, birth asphyxia, maternal methyl mercury exposure, and maternal iodine deficiency
  • CP is restricted to lesions of the brain only; diseases specific to the peripheral nerves of the spinal cord (eg, spinal muscular atrophy, myelomeningocele) or to the muscles (eg, muscular dystrophies), although causing early motor abnormalities, are not considered CP.
  • CP is classified according to resting tone and what limbs are involved. Spastic CP, due to cortex/pyramidal tract lesions, is the most common type and is characterized by spasticity, hyperreflexia, clonus, and an upgoing Babinski reflex.
  • Extrapyramidal or dyskinetic CP is characterized more by abnormal involuntary movements.
  • Functional classification systems generally divide patients into mild, moderate, and severe types (depending on functional limitations).
  • CP is generally considered a static encephalopathy (ie, nonprogressive in nature). However, the clinical presentation of CP changes as children and their developing nervous systems mature.

PERTINENT HISTORICAL FINDINGS/ CLINICAL SYMPTOMS

  • CP diagnosis begins with a history of gross motor developmental delay in the first year of life.
  • Frequently manifests as early hypotonia for the first 6 months to 1 year of life, followed by spasticity.
  • To determine cause patients should have a complete prenatal history, perinatal history, and developmental history.

PERTINENT PHYSICAL EXAM FINDINGS

  • Prior to the formal physical examination, observation may reveal abnormal neck or truncal tone (decreased or increased, depending on age and type of CP); asymmetric posture, strength, or gait; or abnormal coordination.
  • Patients show increased reflexes, indicating the presence of an upper motor neuron lesion. Patients also present with the persistence of primitive reflexes, such as the Moro (startle reflex) and asymmetric tonic neck reflexes (ie, fencing posture with neck turned in same direction when one arm extended and other flexed).
  • Patients with spastic CP evidence spasticity (ie, a velocity-dependent increase in tone) may have tendency to keep the elbow in a flexed position or the hips flexed and adducted with the knees flexed and the valgus and ankles in equinus, resulting in toe walking.
  • Patients with dyskinetic or extrapyramidal CP may have decreased head and truncal tone and defects in postural control and motor dysfunction such as the following: Athetosis, Chorea, Dystonia.
  • Spastic hemiplegia – CP predominantly affecting one side of the body, with upper extremity spasticity more than lower extremity spasticity (eg, right side involved with right arm more so than right leg)
  • Spastic diplegia – CP affecting bilateral lower extremities more than upper extremities
  • Spastic quadriplegia – CP affecting all 4 extremities (full body)
  • Dyskinetic CP (athetoid CP, choreoathetoid CP, and dystonic CP) – CP with extrapyramidal signs characterized by abnormal movements; hypertonicity often is associated
  • Mixed CP – CP with no single specific tonal quality predominating; typically characterized by a mixture of spastic and dyskinetic components
  • Hypotonic CP – CP with truncal and extremity hypotonia with hyperreflexia and persistent primitive reflexes; thought to be rare

DIFFERENTIAL DIAGNOSIS

  • Inherited Metabolic Disorders
  • Traumatic Peripheral Nerve Lesions
  • Hereditary spastic paraplegias
  • Rett syndrome

DIAGNOSTIC EVALUATIONS

  • Laboratory studies are suggested if (1) the clinical history or findings from neuroimaging do not indicate a specific structural abnormality, (2) additional and atypical features are present in the history or clinical examination, or (3) a brain malformation is detected in a child with CP.
  • Considering diagnostic testing for coagulation disorders if a cerebral infarction is seen.
  • If a diagnosis of a hereditary or neurodegenerative disorder is suspected, screening for an underlying metabolic or genetic disorder should be performed. Other blood and urine studies may be considered.
  • Lactate and pyruvate values: Abnormalities may indicate an abnormality of energy metabolism (ie, mitochondrial cytopathy).
  • Thyroid function studies: Abnormal thyroid function may be related to abnormalities in muscle tone or deep tendon reflexes or to movement disorders.
  • Ammonia level: Elevated ammonia levels may indicate liver dysfunction or urea cycle defect.
  • Serum quantitative amino acid and urine quantitative organic acid values: These studies may reveal inherited metabolic disorders.
  • Chromosomal analysis, including karyotype analysis and specific DNA testing: These may be indicated to rule out a genetic syndrome, if dysmorphic features or abnormalities of various organ systems are present.
  • Cranial ultrasonography performed in the early neonatal period can be helpful in medically unstable infants until they are able to tolerate transport for more detailed neuroimaging. Ultrasonography can delineate clear-cut structural abnormalities and show evidence of hemorrhage or hypoxic-ischemic injury.
  • CT scanning of the brain helps identify congenital malformations, intracranial hemorrhage, and periventricular leukomalacia in infants more clearly than ultrasonography.
  • MRI of the brain is the diagnostic neuroimaging study of choice for older children because it defines cortical and white matter structures and abnormalities more clearly than any other method. It also allows for the determination of appropriate myelination for a given age.
  • Head ultrasonography, CT scanning, and MRI may be helpful for diagnosing and monitoring findings of hydrocephalus.
  • In children with spasticity of the legs and worsening of bowel and bladder function, a spine MRI may help identify a tethered spinal cord.
  • EEG is important in the diagnosis of seizure disorders but is not indicated if seizures are not suspected along with CP.
  • Electromyography and nerve conduction studies are helpful when a muscle or nerve disorder is suspected (eg, a hereditary motor or sensory neuropathy as a basis for equinus foot deformities and toe walking).

MEDICAL MANAGEMENT

  • Screening for the following potential CP-associated deficits at the initial assessment is recommended: Mental retardation, Ophthalmologic and hearing impairments, Speech and language disorders, Oromotor dysfunction
  • Various medications may improve spasticity.
  • Numerous medications, although often used off label for age and indication, may relieve the movement difficulties associated with CP. These drugs target dystonia, myoclonus, chorea, athetosis, and spasticity.
  • While antiparkinsonian drugs (eg, anticholinergic and dopaminergic drugs) and antispasticity agents (eg, baclofen) have primarily been used in the management of dystonia, anticonvulsants, antidopaminergic drugs, and antidepressants have also been tried.
  • Anticonvulsants (including benzodiazepines such as diazepam, valproic acid, and barbiturates) have been useful in the management of myoclonus. Chorea and athetosis are often difficult to manage, although benzodiazepines, neuroleptics, and antiparkinsonian drugs (eg, levodopa) have been tried. Benzodiazepines and baclofen are commonly used to manage spasticity.

SURGICAL MANAGEMENT (when applicable)

  • An orthopedist should be consulted for the surgical management of hip dislocation, scoliosis, and spasticity (eg, tenotomy, a tendon-lengthening procedure
  • A neurosurgeon should be consulted for identifying and treating hydrocephalus, a tethered spinal cord, or spasticity.
  • By cutting I-a sensory fibers, selective dorsal rhizotomy decreases spasticity by decreasing reflexive motoneuron activation.
  • Intrathecal baclofen can be administered via a pump implanted by a neurosurgeon.
  • Reconstructive surgery to the upper extremities can restore muscle balance, release contractures, and stabilize joints to improve placement of the hand in space, as well as voluntary grasp, release, and pinch functions.

EMERGENCY MANAGEMENT (when applicable)

  • Not applicable

PATIENT EDUCATION/ MAINTENANCE – PREVENTION

  • Patient management includes a multi-disciplinary approach:
  • A rehabilitation medicine specialist (physiatrist) should be consulted for the evaluation and management of the rehabilitation program (eg, equipment, coordination of therapy, spasticity management).
  • A geneticist should be consulted to evaluate for an underlying genetic syndrome, particularly in the setting of dysmorphic features, multiple organ abnormalities, or a family history of a similar neurological syndrome.
  • A gastroenterologist, nutritionist, and a feeding and swallowing team should be consulted for management of feeding and swallowing difficulties and gastroesophageal reflux and for assessment of nutritional status.
  • A pulmonologist should be consulted for the management of chronic pulmonary disease due to bronchopulmonary dysplasia and frequent or recurrent aspiration.
  • A multidisciplinary learning disability team specializing in children with special needs should be consulted to identify specific learning disabilities, monitor cognitive progression, and guide services through early intervention and school. The child should be evaluated by a communication enhancement center to guide speech and language treatment and the use of communicative devices.
  • Diet: Oromotor dysfunction may require limitations in the texture of food and liquid, feeding only by gastrostomy or jejunostomy tube, supplemental feedings via gastrostomy or jejunostomy tube to increase energy intake, and aspiration precautions.
  • Activity: Regular physical therapy and occupational therapy are crucial. The goal should be to maximize the functional use of limbs and ambulation and to reduce the risk of contractures.

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Dr. Zachary Lahlou

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