Cerebral palsy

• Cerebral Palsy


  A disorder of posture and movement, resulting from a permanent, non-progressive lesion to the immature or developing brain
• First described by William Little


  19th century
• Alternative Terminology


  Static encephalopathy, Little's Disease
• Incidence of Cerebral Palsy: 2-2.5 /1000 in the developed countries
• Cerebral Palsy


  A group of disorders of the development of movement and posture, causing activity limitation that are attributed to non-progressive disturbances that occurred in the developing or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication perception, and/or behaviour, and/or by a seizure disorder
• Cerebral Palsy is reported to be the most common cause of motor deficiency in childhood both in developing and developed countries
• Commonest Causes of Cerebral Palsy in Nigeria and Developing Countries

  • Severe Birth Asphyxia
  • Kernicterus
  • Infections (Intrauterine and Perinatal)
  • Metabolic (Hypoglycaemia)
• Commonest Causes of Cerebral Palsy in Developed Countries

  • Extreme prematurity
  • Inborn Errors of Metabolism
• Causes of Cerebral Palsy

  • Prenatal: Radiation, Developmental anomaly-encephalocoele, Cerebral cysts, Drugs – alcohol, narcotics
  • Perinatal: Anoxia, Birth weight, Asphyxia, Trauma, Age (Gestational) (Prematurity)
  • Postnatal: Infection (Intrauterine), Metabolic – phenylketonuria, hypoglycaemia, Infections –meningitis, encephalitis, Trauma-head injury, Toxins – Kernicterus, Cerebral Hypoxia- FB aspiration, near-drowning
• Early pointers to CP


  • Feeding difficulties, Hypotonia (floppy infant), Head lag, Neonatal seizures
• Later in the 1st year of life


  • Persistence of primitive reflexes e.g. grasp, Moro, sucking, Abnormal posturing- spastic grasp, scissoring, tongue thrusting, persistent thumb adduction, Hand-preference in the 1st year of life, Hypertonia
• Classification of CP


  • Aetiologic: Prenatal, Perinatal, Postnatal
  • Physiologic: Spastic, Dyskinetic, Ataxic, Hypotonic, Atonic, Mixed
  • Topographic: Monoplegia, Hemiplegia, Triplegia, Quadriplegia- Double Hemiplegia, Spastic Diplegia
  • Functional: I - No limitation of activity, II - Slight to moderate limitation, III - Moderate to severe limitation, IV- No useful activity
• Gross Motor Function Classification System (GMFCS)


  A recently developed system which classifies children with CP by their age specific motor activity. It is based on the assessment of severity of CP in children 0-12 years of age based on their functional abilities rather than their limitations. The GMFCS describes the functional characteristics in five levels, from I to V, level I being the mildest.
• GMFCS Levels


  • Level I: Manipulate objects with hands and walk independently, Gets up from sitting without holding unto something, Can climb stairs, Walk indoors and outdoors, climb stairs
  • Level II: Belly crawls, pull to stand on furniture and cruise, Can assume sitting position without assistance, walk with assistive device, Sitting with both hands free, walk short distances without assistive device, Walk indoors or outdoors on level surface only
  • Level III: Can roll and creep forward on stomach, 'w' sit and require adult assistance to assume sitting, Walk with assistive device, Walk indoors or outdoors on level surface with an assistive mobility device
  • Level IV: Can roll independently, Able to roll and creep, can sit when placed, but need both hands on the floor, Sit independently in a chair but minimal hand function, Rely on wheeled mobility, may achieve self-mobility using assistive device
  • Level V: Limited voluntary movements, no head control, Requires adult assistance to roll, All areas of motor functions are limited, Functional limitations in sitting and standing are not fully compensated for through the use of assistive device
• Spastic Diplegia


  Usually seen in preterm infants as a result of the high incidence of IVH & periventricular leukomalacia. Child presents with delayed walking, scissoring gait, commando crawl. Involvement of the UL minimal. IQ usually normal. Seizures are rare.
• Spastic Quadruparesis


  Seen in infants/ children with diffuse brain injury. There is weakness of all 4 limbs, as well as the neck and trunk muscles. Seizures and severe mental retardation are common. Most belong to functional class III & IV, or GMFCS IV & V
• Dyskinetic CP


  Usually found in infants with Bilirubin encephalopathy. Characterized by athetosis & chorea, tongue thrusting, feeding difficulties. There may be associated dental enamel dysplasia, impairment of upward gaze and deafness. Seizures & mental retardation uncommon.
• Spastic Hemiplegia


  Weakness of one UL & the ipsilateral LL. UL involvement more severe than LL. Circumduction characteristic. Seizures in up to 50-60% cases, cognitive impairment in about 25% cases. Hand preference in the 1st year of life is a usual feature.
• Ataxic


  Associated with involvement of the cerebellum. Individual has problems with balance & depth perception. Child experiences difficulties with rapid movements and is often unsteady.
• Associated Deficits in Patients with CP

  • Mental Retardation, Epilepsy, Hearing Impairment, Visual Defects- strabismus, optic atrophy, nystagmus, refractive errors, cortical visual impairment, Speech Defects- delayed, poor-articulation, loss of voice modulation, Learning disability, ADHD (Attention Deficit hyperactive disorder), Failure to thrive, Gait abnormalities, Hip subluxation
• Diagnosis of CP - Early Pointers

  • Delayed motor milestones, Fisting after 5 months of age, Not sitting with support by 8 months, Not walking by 15-18 months, Discrepancies between intellectual and motor development, Persistent or evolving increased or decreased muscle tone, Head lag beyond 6 months of age, Poor trunk control and balance, Opisthotonic posturing and extensor thrusting, Dystonic, Toe walking/scissoring, Abnormal motor or gait patterns
• Diagnosis of CP - Physical Findings

  • Focal abnormalities of movement posture and tone, Differences in functional ability of left/right extremities, Clonus persisting past 12 months, Persistence of primitive reflexes e.g. Moro reflex, Irritability, Easily startled with exaggerated Moro reflex, Excessive crying, Jittery, Sleeping difficulties, Decreased rate of head growth, Poor suck, Delayed feeding milestones, Poor weight gain/failure to thrive
• Diagnosis of CP is mainly clinical, based on a good history and a thorough neurological examination
• Investigations for CP


  • Skull X ray- intracranial calcification, skull defects, Chromosomal studies- to r/o Down syndrome & others, CT scan of the brain - cerebral atrophy, dysgenesis etc, MRI
• Differential Diagnosis of CP


  • Brain tumours, Muscular dystrophy, Down syndrome, Spinal cord injury, Peripheral Neuropathies, Myelomeningocoele
• Management Team for CP

  • Paediatric Neurologist, Ophthalmologist, Audiologist, Speech Therapist, Clinical Psychologist- psycometric testing, Physiotherapist, Occupational Therapist, Special Educator, Social worker, Orthopaedic Surgeon, Behavioural modification
• All children with CP must have hearing & visual assessment
• Management of Specific Problems in CP

  • Seizures: Antiepileptic Drugs, Spasticity: Physiotherapy, Muscle relaxants (benzodiazepine, baclofen, dantrolene, botulinum toxin), Malnutrition: Dietary management, Behavioural problems: Behaviour modification, Respite Care, Tranquilizers (major or minor) are usually not indicated except in extreme/self-injurious behaviour
• Differential Diagnosis of CP

  • Acute poliomyelitis, Muscular dystrophy, Multiple sclerosis, Myelomeningocele, Neonatal brachial plexus palsies, Post polio syndrome, Post traumatic syringomyelia, Spasticity, Stroke (motor impairment), Traumatic brain injury, Neurodegenerative conditions e.g. Tay-Sachs disease, Spinal cord tumour
• Pharmacotherapy for CP


  • Anticonvulsants – To control seizures, Benzodiazepines, Botulinum toxin (botox), Dantrolene sodium, IT Baclofen - To reduce spasticity
• Prognosis of CP is difficult to predict with much accuracy below age 2 years and depends on the associated deficits
• If a child is not sitting up by himself by age 4yr or walking by age 8yr, he will never be an independent walker