ta ogunlesi (fwacp)1 cerebral palsy. ta ogunlesi (fwacp)2 definition cerebral palsy (cp) is also...

TA OGUNLESI (FWACP) 1

CEREBRAL PALSY


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.


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.


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%


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.


AETIOLOGIES

PRENATAL Intra-uterine infections (TORCHES) Intra-uterine hypoxia Antepartum haemorrhage Congenital cerebral malformations Irradiation


AETIOLOGIES PERINATAL Prematurity Asphyxia (Commonest in OOUTH) Cord prolapse Intracranial haemorrhage Hypoglycaemia Kernicterus (2nd leading cause in

OOUTH) Neonatal convulsions (apart from

hypocalcaemia)


AETIOLOGIES

POSTNATALMeningitisEncephalitisHead trauma with intracranial

bleedingProlonged seizures (of any aetiology)Hypertonic dehydrationLead toxicity


PATHOLOGY

CORTICAL ATROPHY Neuronal loss Gliosis Microcephaly Widened ventricles


CLINICAL FEATURES

Cardinal features of CP include:

Delay in motor developmental milestones

Abnormalities of muscle tone Reduction in normal movement Presence of abnormal

movements


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)


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.


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.


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


CLUES TO EARLY DIAGNOSIS Delayed motor milestones

Apathy

Floppiness

Stiffness

Scissoring


CLUES TO EARLY DIAGNOSIS Persistent fisting

Violent startle reactions

Sialorrhoea

Paucity of movements

Strong hand preference in the first year of life


ASSOCIATED PROBLEMS Mental retardation (occurs in ≈ 66% of

cases)

Seizure disorders

Deafness

Blindness, squints, refractive disorders


ASSOCIATED PROBLEMS Orthopaedic problems (hip dislocation,

contractures like toe-walking and scoliosis)

Behavioural disorders (incontinence, constipation etc)

Sialorrhoea

Attention Deficit & Hyperactivity Disorder


DIAGNOSIS

Thorough history (prenatal, perinatal & postnatal)

Physical examination (motor system & sensory organs)

CT scan or MRI of the brain Baseline EEG Audiometry Ophthalmologic evaluation


DIFFERENTIAL DIAGNOSIS

Neurodegenerative diseases Sphingolipidosis Glycogen storage diseases Adrenoleukodystrophies Spinal cord tumours Muscular dystrophies


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.


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.


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).


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)1 cerebral palsy. ta ogunlesi (fwacp)2 definition cerebral palsy (cp) is also...

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