CEREBRAL PALSY CATALINA RUIZ, MD 12/06/12

DEFINITION ETIOLOGY CLASSIFICATION AND CLINICAL FEATURES ASSOCIATED SYNDROMES DIAGNOSIS TREATMENT PROGNOSIS

DEFINITION Difficulty in motor control Non-progressive brain lesion Injury to the brain before it was fully mature (prenatally, perinatally, postnatally) Abnormalities to motor activity and posture

Voluntary movements are supposed to be complex, coordinated and varied. In CP they are uncoordinated, stereotypic and limited (is a spectrum of symptoms, mild to severe) Diagnosis is difficult before 6 months of age, most of the movements are reflex in origin and don’t involve the voluntary motor cortex

ETIOLOGY Prematurity Intrauterine growth restriction Intrauterine infection Antepartum hemorrhage Severe placental pathology Multiple pregnancy Perinatal asphyxia Intracranial hemorrhage Kernicterus Near drowning Trauma Stroke

CLASSIFICATION Based upon the type and distribution of motor abnormalities. Spastic (diplegia, hemiplegia, quadriplegia) Dyskinetic (dystonic, athetoid) Ataxia (simply ataxia, ataxic diplegia)

TO REMEMBER… TONE: is the continuous and passive partial contraction of the muscles. Maneuvers to test muscle tone and normal responses include: Holding the infant under the arms – he/she will assume a sitting position; truncal resistance allows the infant to be easily supported without slipping through the examiner's hands

Passive extension of the legs at the knees – resistance is met at approximately 90º. Pulling the infant from the supine to sitting position – only a slight head lag is expected; the head should wobble in the midline for a few seconds when the sitting position is reached. Holding the infant in horizontal suspension – he/she flexes the limbs, straightens the back, and maintains the head in the midline for a few seconds.

CLASSIFICATION

1. SPASTIC SYNDROMES Spastic hypertonia (clasp-knife) Hyperreflexia caused by hyperexcitability of the stretch reflex Extensor plantar responses Clonus Slow effortful voluntary movements Impaired fine-motor function Difficulty in isolating individual movements Fatigability

SPASTIC

SPASTIC DIPLEGIA Affects both term and preterm infants In preterm infants often is associated with periventricular leukomalacia The lower limbs are affected predominantly Atrophy below the waist occurs in many patients

SPASTIC HEMIPLEGIA Typically affects term infants of normal birth weight The presentation of congenital spastic hemiplegia is variable It is usually caused by a cortical lesion. The arm typically is more affected than the leg

SPASTIC HEMIPLEGIA Suspect when: early hand dominance, reduced movement, or abnormal posturing on one side movement and tone on the affected side typically decrease before tone and tendon reflexes abnormally increase.

SPASTIC QUADRAPLEGIA Most severe form of spastic CP. It usually affects term infants who are small for gestational age. Affected infants typically are severely handicapped. In addition to spasticity, they may have dystonia and feeding and respiratory difficulties because of pseudobulbar palsy

SPASTIC QUADRAPLEGIA Associated findings hip subluxation, contractures, and scoliosis, intellectual disabilities, visual impairment.

2. DYSKINETIC SYNDROMES Neuronal necrosis in the hippocampus, thalamus, basal ganglia, reticular formation, and Purkinje cells of the cerebellum. The neonatal presentation includes encephalopathy characterized by lethargy, decreased spontaneous movement, hypotonia, and suppressed primitive reflexes.

DYSKINETIC SYNDROMES

DYSKINETIC SYNDROMES There are 2 types: Athetoid: movements are athetoid, choreiform, or a combination of both. Dystonic

DYSKINETIC SYNDROMES ATHETOID MOVEMENTS: slow, smooth, writhing movements that involve distal muscles athetosis – YouTube Athetosis is most apparent during reaching, as the fingers extend and abduct.

DYSKINETIC SYNDROMES CHOREA: rapid, irregular, unpredictable contractions of individual muscles or small muscle groups that involve the face, bulbar muscles, proximal extremities, and fingers and toes. Stress, excitement, or fever may exacerbate the chorea CHOREA

DYSKINETIC SYNDROMES DYSTONIC: repetitive, patterned, twisting, and sustained movements of the trunk and limbs that may be either slow or rapid. DYSTONIA The muscle tone typically is normal or hypotonic, especially in early childhood. Infants tend to have retention of primitive reflexes, involuntary grimacing, a tendency to drool, and delayed psychomotor development.

DYSKINETIC SYNDROMES The involuntary movements evolve with time and may not be apparent until the child is two to three years of age. Unlike spastic CP, contractures usually do not develop, unless they are positional. Patients often have more than one form of involuntary movement, and the types may overlap in some cases. The extent of dysarthria and motor and intellectual disability are variable.

3. ATAXIA Although ataxia indicates an incoordination of cerebellar or sensory origin, ataxic CP represents a widespread disorder of motor function. Ataxia The etiology of ataxic CP is heterogeneous. Most cases are caused by early prenatal events.

ATAXIA Most patients have congenital hypotonia. Motor milestones and language skills typically are delayed. Ataxia usually improves with time. Speech, which is related to intellectual ability, typically is slow, jerky, and explosive. The diagnosis of ataxic CP is made by exclusion.

ASSOCIATED CONDITIONS INTELLECTUAL DISSABILITY: 52% of patients. Severity correlates with the extent of motor handicap. Children with spastic quadriplegia are typically the most severely affected PSYCHIATRIC DISORDERS: emotional lability, poor attention and vigilance, and obsessive-compulsive traits

ASSOCIATED CONDITIONS EPILEPSY: 45% of patients. Most common in patients with spastic quadriplegia and acquired hemiplegia. The onset of seizures is typically during the first two years of life. Partial seizures with secondary generalization are the most common type. Intellectual disability is more common in CP patients with seizures than in those without seizures

ASSOCIATED CONDITIONS VISUAL DISORDERS: strabismus and refraction errors are the most common ones. Ambliopia and visual fields defects can also be present. Low visual acuity may be due to cortical impairment.

ASSOCIATED CONDITIONS SPEECH IMPAIRMENT : aphasia and dysarthria (38%). Abnormal function of oropharyngeal muscles and lack of coordination of breathing patterns contribute.

ASSOCIATED CONDITIONS HEARING IMPAIRMENT: 10-20%. It is most common in those with very low birthweight or severe hypoxic-ischemic insults. Sensorineural hearing loss is also a feature of CP caused by kernicterus.

ASSOCIATED CONDITIONS GROWTH FAILURE: mostly due to poor nutrition. Poor nutritional status is caused by both inadequate intake and gastrointestinal abnormalities

ASSOCIATED CONDITIONS PULMONARY DISEASE: leading cause of death. Poor coordination of respiratory muscles and chest deformity. Recurrent aspiration (reflux), restrictive disease (scoliosis).

ASSOCIATED CONDITIONS ORTHOPEDIC DISORDERS: subluxation, dislocation, and progressive dysplasia of the hip, foot deformities, and scoliosis. Osteopenia.

ASSOCIATED CONDITIONS URINARY DISORDERS: dysfunctional voiding symptoms, including enuresis, frequency, urgency, and stress incontinence. Neurogenic bladder and incontinence.

DIAGNOSIS Combination of findings, including motor delay, neurologic signs, persistence of primitive reflexes, and abnormal postural reactions. Infants with an abnormal obstetric or perinatal history may be at increased risk to develop CP, and should be monitored closely.

DIAGNOSIS CLUES: Psychomotor delay Abnormal oromotor or oculomotor patterns Hypotonia/hypertonia Hyperreflexia, clonus

DIAGNOSIS

DIFERENTIAL DIAGNOSIS CP is a diagnosis of exclusion. Neurodegenerative diseases Inborn errors of metabolism Neuromuscular or movement disorders Neoplasm

TREATMENT Multidisciplinary treatment Psychological development, communication, and education are priorities in the management . Interventions should be directed to maximize the quality of life.

TREATMENT PT/OT Botulism toxin Antispastic drugs (Dantrolene, benzos, Baclofen) Surgery (muscle-tendon, hip) Constraint-induced movement therapy (CIMT) Gastrostomy feeding

TREATMENT Motorized wheelchairs, switching devices used to activate communication systems, voice-activated computers Braces Psychological support

PROGNOSIS The majority of children with CP survive to adulthood. Survival of adults with CP also depends upon the degree of disability. The prognosis for survival and motor function in children with CP is highly variable, and depends on the severity and type.

PROGNOSIS The prognosis for walking is poor in children who do not achieve head balance by 20 months, retain primitive reflexes or have no postural reactions by 24 months, or do not crawl by approximately five years of age. The prognosis for walking is good in children who sit by two years and crawl before 30 months of age.

REFERENCES UP TO DATE NELSON PEDS IN REVIEW