CEREBRAL PALSY

DEFINITION A disorder of movement and posture resulting from a non-progressive injury to the developing brain.

NEW DEFINITION CP describes a group of disorders of development of movement and posture, causing activity limitation that are attributed to non- progressive disturbances that occurred in the developing fetal or infant brain. The motor disorder of CP are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behaviour and/or by seizure disorder”

EPIDEMIOLOGY Incidence is about per 1000 live births- Developed world and in developing nations. CP accounted for 16.2% of new referrals to child neurology clinic % of injury occur antepartum, 5% intrapartum and about 15% after birth.

All Races are affected Incidence is higher in males; M: F ratio 1.33:1 Lower socioeconomic status

AETIOLOGY Unknown in about 50% of cases. Prenatal/congenital causes 80-90%  Cerebral malformations & dysgenesis.  Neurological damage as a result of –  Congenital/ intrauterine infections  Placental factors poor brain oxygenation  Vascular factors( CHD,DIC, Coagulopathies)  Effects of environmental toxins (infarcts

Intra-partum/Postnatal causes Birth asphyxia Kernicterus Infections(viral & Bacterial) Post surgical vascular complications Asphyxia due to aspiration or drowning Traumatic brain injury: - Accidental 20% - Non Accidental 80%

CLASSIFICATION Edinburgh Spastic 60-70% - Spastic hemiplegia - Spastic Diplegia - Spastic Quadriplegia Athetoid/ Dyskinetic 10-20% Ataxic 10-15% Mixed 10% -- - mixture of diff types, eg spastic & Athetoid.

 Pathophysiologic classification Insults resulting in neuronal loss can be  Cortical (pyramidal), resulting in spasticity  Basal ganglial (extrapyramidal), resulting in abnormal movements such as choreoathetosis  Cerebellar, resulting in hypotonia, or  Mixed

Pathogenesis and Clinical Features Spastic CP – most common, up to 75%. Based on topographic distribution of motor involvement the following  Spastic Diplegia – more of lower limb involvement; most common; associated with prematurity  Spastic Hemiplegia – more of arm involvement  Spastic Monoplegia – result of very mild hemiplegia  Spastic Quadriplegia – all four limbs  Double hemiplegia – quadriplegia with more of arm involvement  Triplegia – rare, results from milder and very asymmetric double hemiplegia (sparing one leg) or milder asymmetric diplegia (sparing one arm)

Spastic Hemiplegia – results from stroke- like events with focal cortical infarction(haemorrhagic infarction, infection, thrombophilic disorders) --↓ spontaneous activity on involved side with hand preference, --difficulty in hand manipulation by 1yr, -- walking is delayed to between 18–24 months, with circumductive gait --Growth arrest esp in hand and thumb nail (involvement of contralateral parietal lobe) --Spasticity of affected extremity, ↑ tendon reflexes, ankle clonus, positive Babinski response. --One third have seizure disorder, --approx 25% have cognitive impairment

Spastic Diplegia – usually results from periventricular leukomalacia (PVL; seen in prematurity, ischaemia) --1 st noticed when infant begins to crawl (uses arms more--commando crawl, dragging feet along) --Scissoring posture when suspended at axillae --Spasticity in legs with brisk reflexes, ankle clonus and bilateral Babinski reponse --Good prognosis for normal intellect

Dyskinetic (Extrapyramidal or Choreoathetoid) CP  follows birth asphyxia, kernicterus;  pathology is in basal ganglia  Less common than spastic CP Hypotonia initially with Head lag; later tone increases with rigidity and dystonia; involuntary movts Speech is typically affected (involvement of oropharyngeal muscles), also sensorineural hearing loss UMN signs are absent, seizures are uncommon, intellect is usually preserved

Spastic Quadriplegia – most severe form of CP, all four limbs affected; associated with severe PVL and cortical encephalomalacia Mental retardation and seizures common Swallowing difficulty. Decreased spontaneous movements, increased tone and spasticity all limbs, brisk reflexes and positive Babinski response Speech and Visual problems May have evidence of athetosis

Hypotonic CP Occurs rarely Results from cerebellar insult most children progress to other CP subtypes. Mixed CP Occurs when the child displays a combination of features, such as spasticity and choreoathetosis

Other Clinical Features Mental retardation – not in all CP patients. Spastic diplegic CP is characterized by normal cognition while Spastic quadriplegic CP has greater MR Factors associated with MR include -severity of motor deficit, -epilepsy, and -cortical abnormalities on neuroimaging

Epilepsy – seen in 36% of pts with 70% manifesting in 1 st yr of life (more of quadriplegic and hemiplegic than diplegic CP). Usually focal seizures ± secondary generalization Poor growth – due to difficulty in feeding from pseudobulbar palsy Bladder and Bowel dysfunctions Bladder: urinary incontinence with UTIs Bowel: constipation due to poor feeding, reduced water intake and immobility

Visual and Hearing problems Orthopaedic problems e.g. equinovarus deformity (tip-toe walking), hip dislocation (from severe spastic diplegia), flexion contracture of knees and elbows Speech abnormalities

MANAGEMENT OF CP History is important Detailed physical/neurological exam Investigation : EEG;FBC + Bld film; Urinalysis & urine m/c/s; CT Scan; MRI; Hearing & Visual Assessments

 DIAGNOSIS Diagnosis- difficult clinically in the first year:  Limited variety of volitional movt for evaluation  Substantial myelination takes months to evolve delaying the clinical picture of hypertonia/hyper reflexia

TREATMENT Not Curable Treatment is symptomatic and entails Multidisciplinary approach Various forms of therapy help affected persons function and live more effectively The earlier therapy begins the better Nutritional support: About 30% are undernourished. Many show growth delays. Early NG feeding & later Gastrostomy tube feeding may lead to improved growth

Give antiepileptic drugs for seizures eg carbamazepine If there is generalized spasticity, give baclofen, diazepam or dantrolene and if localized give injection botulinium toxin (Botox) for 3 – 6 mths Physical therapy[Physiotherapy] --Aimed at encouraging the patient to build a strength base for improved gait & volitional movt -- Stretching program to limit contractures

 Occupational therapy:  Helps maximize their function,and adapt to their limitation  To live as independently as possible  Speech therapy:  Usually starts before the child begins school  Helps to control the muscles of the mouth & jaw  Helps to improve communication

 Orthotic intervention using ankle-foot orthoses aimed at prevention and/or correction of deformities  Provision of support & facilitation of skill development  Improvement of gait  Surgery usually the last resort -lengthening of tendons -Rhizotomy[cutting of nerve roots]

PREVENTION Most of the causes of CP [62%] are preventable and the 5 levels of prevention are involved. Mothers should be taught to ensure optimal wellbeing prior to conception Good obstetric care should be encouraged Anti-D immunoglobulin for Rh-ve mothers Vaccination against some IU infections Proactive treatment to minimize the effects of perinatal brain injury.

High risk pregnant women should be identified early and referred for appropriate management Screening children at birth for G6PD deficiency Good NICU[Neonatal Intensive Care Unit] for those children who have developed birth asphyxia, severe jaundice and LBW Maintaining a good referral pediatric service Physiotherapy Rehabilitation of pts

PROGNOSIS Actual brain damage does not worsen but symptoms can become worse overtime due to subdural haemorrhage. The collaborative perinatal project found that 50% of individuals diagnosed with CP and 66% of children diagnosed with spastic diplegia “outgrew” findings by 7yr of age The ability to sit independently at 2yrs of age is predictive of future ambulation Regarding life expectancy and mortality rates, the type and severity of disability and feeding skills are major determinants

Overall, the probability of reaching the age of 20yrs in a child with severe CP is 50% Respiratory infections, aspirations and epilepsy are the leading causes of death Newer therapeutic advances and the degree of parental care have a strong influence on the length of survival of these children