CEREBRAL PALSY TA OGUNLESI (FWACP)

DEFINITION CEREBRAL PALSY (CP) is also known as LITTLE’S DISEASE or STATIC ENCEPHALOPATHY. It is defined as a disorder of posture and movement which is caused by a permanent and non-progressive insult to the developing brain. The permanent brain lesion that results in CP affects other brain functions apart from the motor area resulting in speech, auditory, visual and mental deficits but these are NOT part of the definition of CP. TA OGUNLESI (FWACP)

CP results from an abnormality in or injury to the cerebrum — the largest area of the brain, which controls sensation and voluntary motor function. Although CP affects movement, the underlying problem originates in the brain, not in the muscles themselves. The cerebral lesion is STATIC but the movement disorder may change with time. TA OGUNLESI (FWACP)

EPIDEMIOLOGY The Collaborative Perinatal Project in US, in which approximately 45,000 children were regularly monitored from pregnancy to the age of 7yr, reported the prevalence of CP to be 4/1,000 live births. The prevalence of Cerebral Palsy among children attending Paediatric Neurology Clinic in OOUTH between 1996 and 2000 was 50.3% TA OGUNLESI (FWACP)

AETIOLOGIES These vary in different parts of the world depending on the spectrum of prenatal, perinatal or postnatal insults to which the fetal and neonatal brains are exposed. Intra-uterine infections and extreme prematurity are the commonest causes of CP in the developed world, perinatal hypoxia arising from poor neonatal resuscitation at birth is the most common cause in the developing world. TA OGUNLESI (FWACP)

AETIOLOGIES PRENATAL Intra-uterine infections (TORCHES) Intra-uterine hypoxia Antepartum haemorrhage Congenital cerebral malformations Irradiation TA OGUNLESI (FWACP)

AETIOLOGIES PERINATAL Prematurity Asphyxia (Commonest in OOUTH) Cord prolapse Intracranial haemorrhage Hypoglycaemia Kernicterus (2nd leading cause in OOUTH) Neonatal convulsions (apart from hypocalcaemia) TA OGUNLESI (FWACP)

AETIOLOGIES POSTNATAL Meningitis Encephalitis Head trauma with intracranial bleeding Prolonged seizures (of any aetiology) Hypertonic dehydration Lead toxicity TA OGUNLESI (FWACP)

PATHOLOGY CORTICAL ATROPHY Neuronal loss Gliosis Microcephaly Widened ventricles TA OGUNLESI (FWACP)

CLINICAL FEATURES Cardinal features of CP include: Delay in motor developmental milestones Abnormalities of muscle tone Reduction in normal movement Presence of abnormal movements TA OGUNLESI (FWACP)

TYPES OF CEREBRAL PALSY SPASTIC – upper motor neurone pattern of weakness, hypertonia, hyper-reflexia & extensor plantar reflex. May be lead-pipe or clasp-knife in form. Most common type – about 75% of cases. Hemiplegia (one side; upper limb more affected than lower limb) Diplegia (both lower limbs more affected than upper limbs; associated with prematurity) Quadriplegia (all limbs equally affected) ***** Double hemiplegia Paraplegia (very rare) TA OGUNLESI (FWACP)

TYPES OF CEREBRAL PALSY ATHETOID (DYSKINETIC) –continuous, slow, writhing movements, due to basal ganglia damage from either kernicterus or asphyxia. Usually associated with auditory and speech defects but intellect is often preserved. About 20% of cases. ATAXIC (EXTRAPYRAMIDAL) - lack of balance reactions, intention tremors, broad based gait, in-coordination of hand movements. Associated with prenatal cerebellar damage. Least common type. TA OGUNLESI (FWACP)

TYPES OF CEREBRAL PALSY HYPOTONIC- Hypotonia is present at rest but features of UMNL may manifest. Usually severely mentally retarded. Uncommon. MIXED - a combination of different types. TA OGUNLESI (FWACP)

FUNCTIONAL CLASSIFICATION CLASS I – no limitation of activity CLASS II – slight to moderate limitation CLASS III – moderate to severe limitation CLASS IV – no useful physical activity TA OGUNLESI (FWACP)

CLUES TO EARLY DIAGNOSIS Delayed motor milestones Apathy Floppiness Stiffness Scissoring TA OGUNLESI (FWACP)

CLUES TO EARLY DIAGNOSIS Persistent fisting Violent startle reactions Sialorrhoea Paucity of movements Strong hand preference in the first year of life TA OGUNLESI (FWACP)

ASSOCIATED PROBLEMS Mental retardation (occurs in ≈ 66% of cases) Seizure disorders Deafness Blindness, squints, refractive disorders TA OGUNLESI (FWACP)

ASSOCIATED PROBLEMS Orthopaedic problems (hip dislocation, contractures like toe-walking and scoliosis) Behavioural disorders (incontinence, constipation etc) Sialorrhoea Attention Deficit & Hyperactivity Disorder TA OGUNLESI (FWACP)

DIAGNOSIS Thorough history (prenatal, perinatal & postnatal) Physical examination (motor system & sensory organs) CT scan or MRI of the brain Baseline EEG Audiometry Ophthalmologic evaluation TA OGUNLESI (FWACP)

DIFFERENTIAL DIAGNOSIS Neurodegenerative diseases Sphingolipidosis Glycogen storage diseases Adrenoleukodystrophies Spinal cord tumours Muscular dystrophies TA OGUNLESI (FWACP)

MANAGEMENT Early diagnosis and institution of treatment is desired. Requires multi-disciplinary approach (paediatric neurologist, paediatric nurse, physiotherapist, speech therapist, orthopaedic surgeon, social worker, educator). Parents should be taught to address the peculiarities of daily activities such as feeding, dressing, bathing and playing in ways that limit the effects of abnormal muscle tone. TA OGUNLESI (FWACP)

MANAGEMENT Use of adaptive equipments like walkers, poles, and standing frames, motorized wheelchairs, special feeding devices and customized seating arrangements. Drugs (Dantrolene sodium 0.5mg/kg/ dose bd; Baclofen 10-15mg/day, Diazepam 0.3-0.5mg/kg/dose bd) to reduce spasticity. Direct injection of Botulinum toxin into the affected muscle is still undergoing trials. TA OGUNLESI (FWACP)

MANAGEMENT Hearing and vision aids Orthotics like braces and splints Enrolment in special schools Communication skills may be enhanced by the use of special devices. Surgical procedures like release of contractures and dorsal rhizotomy (transection of dorsal nerve roots supplying spastic muscles). TA OGUNLESI (FWACP)

PREVENTION Prevent birth asphyxia Early diagnosis and prompt intervention in neonatal jaundice. Prevent intra-uterine infections with immunization with MMR NICU for extremely premature infants has been shown to increase the survival of these vulnerable babies & the incidence of CP. TA OGUNLESI (FWACP)