Cerebral palsy (CP)

Chronical disorder, ranging in severity, characterized by abnormal muscle tone, posture, and movement.

Etiology

maternal factors:

alcohol consumption, smoking, obesity, and infections during pregnancy neonatal factors:

perinatal hypoxia-ischemia
prematurity –due to periventricular leukomalacia (PVL), intraventricular hemorrhage (IVH), and/or bronchopulmonary dysplasia (BPD)
Intrauterine infections like cytomegalovirus, syphilis, varicella virus, and toxoplasmosis
Kernicterus − typically resulting in choreoathetosis CP
congenital abnormalities, particularly structural central nervous system abnormalities
others including intrauterine growth restriction, intrauterine infection, antepartum hemorrhage, severe placental pathology, multiple pregnancy

causes of the older child:

Stroke eg in congenital heart disease, prothrombotic disorder, sickle cell disease, vasculopathy, or metabolic disorder. Stroke typically results in spastic hemiparesis
Trauma
Severe hypoxic events such as near-drowning
Sepsis or meningitis, cerebral malaria

Clinical presentation

specific CP syndromes recognized after five years of age, although suggestive signs and symptoms may be present in infancy. Typical history includes poor feeding in the neonatal period, irritability, poor sleep, vomiting, difficult to handle and cuddle, and poor visual attention. Developmental reflexes delay in disappearance. Motor tone in the extremities may be normal, decreased, or increased. Persistent or asymmetric fisting, abnormal oromotor patterns include tongue retraction and thrust, tonic bite, oral hypersensitivity, and grimacing. Poor head control. Not archiving motor milestones (e.g., six motor milestones - roll prone to supine, roll supine to prone, sit with support, sit without support, crawl, and cruise)

Classification

Spastic syndromes: symmetric or asymmetric, involve one or more extremities. Involuntarily increased muscle activity or movement patterns like spastic hypertonia, if very severe form rigid in flexion or extension, hyperreflexia, clonus, slow effortful voluntary movements, impaired fine-motor function, difficulty in isolating individual movements, fatigability and weakness. Often leading to contractions.
Forms:

spastic diplegia - if mild relatively good hand function, in severely affected patients, upper limb function compromised, sensory loss, associated involuntary movements, and intelligence. degrees of flexion at the elbows and knees, and flexion, adduction, and internal rotation of the hips, equinovalgus or calcaneovarus deformity of the foot.
Spastic hemiplegia – upper limb typically more affected than lower limb. Usually vascular origin.
Spastic quadriplegia (tetraplegia) - typically severely handicapped, usually associated impairments (severe intellectual disability, communication and visual impairment, epilepsy, feeding difficulties, and/or pulmonary disease)

Dyskinetic syndromes: involuntary movement

Choreoathetosis: rapid, irregular, unpredictable contractions of individual muscles or small muscle groups that involve the face, bulbar muscles, proximal extremities, and fingers and toes. Athetosis consists of slow, smooth, writhing movements that involve distal muscles. dyssynergia (antagonistic action) of opposing muscle groups, such as flexion and extension or pronation and supination.
Dystonia: pyramidal signs and anarthria. Sudden involuntary increase in tone that affects both flexor and extensor muscles. The limbs become stiff during attempted movement or with emotion.

Ataxic CP (rare): Motor milestones and language skills are delayed. Ataxia usually improves with time. Speech is slow, jerky, and explosive

Differential Diagnosis

Muscle distrophie, spinal muscle atrophia, M. Wilson, Chorea Huntington, tethered cord syndrome, genetic syndromes (Prader-Willi, Angelman Syndrome)

Important points in history

Birth history, neonatal jaundice, infections, trauma, seizures, milestones

Important points on examination

full neurological examination, head circumference, dysmorphia

Associated conditions

Pain (50 to 75 percent), intellectual disability (50 percent), speech-language disorders (40 percent, aphasia and dysarthria, 25 percent are nonverbal), epilepsy (25 to 40 percent, onset of seizures during the first two years of life, usually partial seizures with secondary generalization), visual impairment (30 percent), behavior disorder (25 percent), bladder control problems (30 to 60 percent), sleep disorder (20 percent), hearing impairment (10 to 20 percent), growth failure - primarily due to poor nutrition, GI problems: Chronic constipation (60 to 70 percent), gastroesophageal reflux and/or vomiting (approximately 50 percent), swallowing disorders (approximately 20 percent), pulmonary disease (recurrent aspiration, scoliosis, respiratory muscle dyscoordination and weakness – result in uneffective cough and clearance of pulmonary secretions), orthopedic disorders (subluxation, dislocation, and progressive dysplasia of the hip, foot deformities, and scoliosis, osteopenia), Urinary disorders (enuresis)

Treatment

Education of child and guardians

Nutrition – Moyo Assessment

Physiotherapy for limbs and chest as well as sucking and swallowing drills

Drugs:

Against spasticity:
- Baclofen: 1 (-2) mg/kg TDS. Side effects: confusion, sedation, hypotonia, ataxia, paresthesias, and nausea. Seizures if drug discontinued abruptly
- Diazepam
Antiepileptics – see Epilepsy protocol

consult orthopedics if severe deformities or acute hip disorders

social and psychological support by umodzi