Cerebral Palsy(1)

CEREBRAL PALSYDr. Maninder S. Dhaliwal

Define Is defined as a :Persistent but not unchanging Disorder of movement, tone and postureDue to non-progressive defect/lesionOf immature brain ( fetal life, infancy, childhood)

( immature brain cut off take as 5 yrs AAP)

5) Commonly associated with a spectrum of developmental disabilities such as Mental retardation (60%)Epilepsy (33%)Visual , hearing (deafness-10%) and speech defectsStrabismus(50%)Cognitive dysfunctionSensory problemsEmotional and behavioral problems.

EXCLUDING progressive pathology and lesion of spinal cord.

INCLUDING non-progressive genetic d/s or cong malformation

CLASSIFICATIONS TOPOGRAPHICMONOHEMIDIPLEGIAQUADRIDOUBLE HEMIPLEGIATRIPLEGIAPHYSIOLOGYSPASTICEXTRAPYRAMIDALATAXICMIXEDATONIC

FUNCTIONAL

CLASS 1 NO limitation of activityCLASS 2 Slight limitation CLASS 3 Moderate limitationCLASS 4 No useful physical activity

Site of brain injury

Cortical Sub corticalPeriventricularBasal gangliaCerebellumBrain stemPathological

Periventricular leucomalacia spastic diplegiaStroke in utero - hemiplegiaMultifocal encephalomalacia -quadriplegiaCerebellar - ataxicBasal ganglia, thalmus, putamen - dyskinetic

Severity Motor development quotient :

= motor age / chronological age 100

>70 : minimal deviancy without delay50-70 : mild motor disorder

Etiological PrenatalI, iron def.,poor nut.Inf, UTI, high feverChorioamniotisHTN, DMTeratogensPoor ANCLOW SESTwinsFetal vasculopathyPerinatalBirth asphyxiaPremature / LBWIUGRHyperbilirubenemiaIVHSepsis, pneumonia, meningitisDevelop. malformationPostnatalCNS infectionsHead injuriesSeizuresHypoxic damageHyperpyrexia damage

Early markers of CPSLOW head growthPoor head controlEye roving eyes, poor hand regard, persistent squint.Ear lack of auditory responseIrritability, seizures, poor suck, poor quality of sleep.Extreme sensitivity to light

Cortical thumb beyond 8 weeksHandedness before 2 yrsPaucity of limb movementsScissoring of lower limbsToe walkingAbnormal tonePersistence of primitive reflexes or failure to acquire postural reflexesStereotypic abnormal movementsLack of alertness

Differential diagnosis In the early infancy when the child is in hypotonic phase, neuromuscular conditions like myopathies may cause diagnostic confusion.

Children with mental retardation may have hypotonia but do not have abnormal motor patterns or postures

Neurodegenerative conditions which have onset in early infancy such as Tay-Sach disease, Krabbe's disease, and Metachromatic Leucodystrophy can mimic CP.

The progressive course of these conditions can be ascertained on the basis of history, and relevant investigation can confirm the diagnosis.

Dopa-responsive dystonia and organic aciduria like glutaric aciduria may look like dystonic CP.

Assessment of General Health

Growth, and nutritional disorders Frequent respiratory tract infections are common because of ineffective cough reflex. Facial dysmorphism and other congenital anomalies should be noted. Skin should be inspected for neurocutaneous stigmata. Head circumference should be noted and plotted .

Reliable measures of length may be difficult to obtain due to concomitant contractures or scoliosis.

Alternate measures to length such as segmental measures of upper arm and lower leg are sometimes used.

Skinfold thickness is a useful and a less cumbersome method of assessing nutritional status.

Neurological Evaluation

The history of previous developmental milestones should be obtained for all domains of development i.e. gross & fine motor, cognitive, speech and language and socialization.

A thorough neurological evaluation should be performed which includes assessment of cranial nerves, posture, muscle tone of extremities, trunk and neck, deep tendon reflexes; postural response and primitive reflexes .

Physical examination should include the observation of child in prone, supine, sitting, standing, and if appropriate in walking and climbing positions.

This is followed by assessment of the current level of functioning in all these domains and assessment of self help and adaptive skills in daily activities such as feeding, dressing, brushing teeth .

Disorder in Movement and Posture Among the most clinically useful primitive reflexes are Moro, Tonic labyrinthine and ATNR

Postural reactions are sought in each of 3 categories: righting, protection and equilibrium.

Muscle Tone Abnormality of tone is an integral part of CP.Hypertonia in CP may be purely due to spasticity (pyramidal ) or else due to dystonia (extrapyramidal).

Assessment of Cognition and BehaviorMetal retardation was found the commonest associated problem in children with CP

Conventional tests of intelligence may prove erroneous in children with CP because of motor and communication deficits.

Age appropriate non-verbal intelligence tests have to be administered for this purpose.

Assessment of Vision and Hearing In children with hearing impairment with associated microcephaly and congenital heart disease should be looked for other stigmata of TORCH infection.

In cases with dyskinetic CP, presence of hearing impairment may point to kernicterus as a cause of CP.

Sensorineural hearing loss is a prominent feature of CP due to Iodine deficiency in endemic areas.

Assessment of Speech and LanguageThese may be due to hearing impairment, cognitive deficits, or oromotor dysfunction

Difficulty in communication by language or gestures further compound behavior problems.

Assessment of Feeding and NutritionOromotor dysfunction, inability to self feed , and inability to request for food due to communication disorder result in feeding problems and poor nutritional status in children with CP .

Gastro-esophageal reflux or choking/coughing while feeding which may further cause aversion to food .

Child should also be assessed for tongue thrust or tonic bite, hypersensitivity to touch, drooling of saliva and dental hygiene.

Orthopedic Problems Hip subluxation, scoliosis, equinus deformity, and contractures of hamstring muscles and tendoachilles.

Reduced bone density and propensity to fractures with trivial trauma is common

Equinus deformity is the most common musculoskeletal abnormality in patients with CP.

It is due to fixed or spastic contracture of gastrocnemius and causes the typical tip toe or toe heel gait in children with CP.

Epilepsy: Epilepsy is more common in children with CP. In a population based study 38% of children with CP had epilepsy

Children with CP caused by CNS malformations, CNS infection, and grey matter damage have been reported to show a higher frequency of epilepsy than children with CP of other aetiology, and also had less chance of becoming seizure-free

Role of NeuroimagingNeuroimaging (MRI preferred to CT) is recommended in children with cerebral palsy in order to establish structural brain abnormality which may further help in finding the etiology and giving prognosis

Disabling conditions be evaluated on multiple axes-

Pathophysiology (underlying disease);Impairment (clinically observable abnormality) ;Functional limitations (effect on task performance);Disability (effect on daily living) and Societal limitations (effect on life time opportunities).

Since CP is a changing disorder it is evident that some limitations may not be evident early in life but manifest in the school age or later.

Assessment of Home Situations: Evaluation is not complete without the assessment of the home situation such as family size, financial resources and family support.

Child rearing conditions that support and enrich early development can compensate for many biological deficits.

On the other hand, poverty, illiteracy in parents, large family size, frequent change in residence, non-availability of special rehabilitation centers may deprive the child of appropriate care.

The hemiplegic type of CP has the best prognosis for walking with 95% walking by 3 years compared to 40% of other groups.

Age of sitting is a good guide to prognosticate about walking. A child who is able to sit unsupported at 2 years will eventually be able to walk. On the contrary, a child whose sitting is delayed beyond 3 years has remote prospects for functional outdoor walking

Management - The pediatrician's initial role consists of making a correct diagnosis,determining the etiology, and identification of the type, extent and severity of the neuromotor deficit as well as of associated problems

(i) Breaking the news

ii) Comprehensive assessment a multidisciplinary team comprising of a neuro-developmental pediatrician as the team-leader, physiotherapist, occupational therapist,clinical psychologist, speech pathologist, orthopedic surgeon,otorhinolaryngologist, ophthalmologist, teacher, play therapist and social worker is required, preferably under one roof.

Physiotherapy (P.T.) -P.T. especially when started early in life, is helpful in promoting normal motor development, and preventing deformity and contractures. In the young child it aims at reducing abnormal patterns of movement and posture and promoting the normal ones so as to enable the child to gain maximal functional independence. A number of techniques have been used for this purpose. The neurodevelopmental Bobath technique is commonly used.

It consists of guiding the child through normal sequences of motor development, inhibition of primitive and abnormal reflexes, re-inforcement of normal postural reflexes and facilitation of normal movements.

Occupational TherapyThe role of P.T. and O.T. are so closely linked that they could infact be considered together.The occupational therapist is usually better trained to advise on activites of daily living like feeding, bathing, dressing, toilet training etc, and the equipment needed to facilitate these. Co-ordination and sensory-perceptual integration can be taught and multisensorial stimulation provided through peg board, blocks and other toys of different colours, textures, sizes and shapes, and producing different sounds

Play Therapy: It is the use of a natural activity with a young child, to help him consolidate the levels of development that he has reached and encourage him to move on, to the next level.

Parents are taught to break down each activity into its simplest components and make the child practice it in a real life situation. It then is not considered as an 'exercise' but becomes a way of life.

Assistive and adaptive devices : Various simple modifications like angled spoons, two handled cups etc can be made to help the child. Old stools and boxes can be adapted to provide support during sitting Parallel bars can be constructed with logs of wood to help gait training. Standing frames and prone boards are a useful intermediate stage in mobilization.

A number of high technology devices like programmed wheel chairs, electronic feeding devices, various access systems, computerized speech systems and cochlear implants are available

Splints, Casts and Calipers: Specially designed shoes, ankle-foot orthoses (AFO) and calipers AFOs, are particularly useful in children with spastic diplegia who have dynamic spasticity with tendo- achilles (TA) tightening.

Management of Spasticity: Proper P.T. given regularly considerably reduces spasticity and improves function. (i) Drugs:Baclofen - acts at the level of spinal cord neurons and enhances GABA activity. It is commonly used in a starting dose of 1.25 - 2.5 mg BD orally and increased gradually upto a maximum of 30 mgm/day, monitored by a clinical response. It is not recommended in children with seizures as it may provoke them. (

ii) Diazepam- a small dose given half an hour before PT is effective in some cases especially where anxiety increases spasticity. Its disadvantage is that it may cause unacceptable drowsiness. (iii) Tizanidine an alpha 2 adrenergic agent and (iv) Dantrolene sodium which acts on calcium channels have also been used, but experience with them is limited.

Baclofen has also been used intrathecally using implantable infusion pumps. It may be helpful in cases of severe spasticity or disabling total body dystonia

Botulinum Toxin: (BTA): is derived from Claustridium botulinum.

It causes muscle relaxation by blocking the release of acetylcholinesterase, with loss of motor end plates. As affected nerve roots sprout to form new junctions, the relaxing effect reverses over 3-6 months. It is more often used in children with spastic diplegia.

Surgery: Surgery is useful in some children with spasticity, especially where mainly the lower limbs are involved.

Tendon lengthening and transfer and arthrodesis are some of the procedures commonly performed. Generally multilevel surgery is required and is done after 8 years of age. Simultaneous availability of intensive physiotherapy is essential.

Dorsal rhizotomy which involves selective resection of posterior nerve roots from L 2_ to S 2

It may be helpful in children with severe lower limb spasticity, with sufficient trunk control and some form of forward locomotion.

Its advantage must be weighed carefully against the sensory losses that may occur after the procedure.

Relief of athetosis and dystonia - is difficult occasionally levo-dopa for severe athetosis and carbamezepine for dystonia may be helpful. Thalamotomy for athetoid CP, stereotactic dentatomy and chronic cerebellar stimulation via implanted electrode .

Management of Associated Problems.

Parent Counselling : This is one of the most important aspects because parents are pivotal in the management of their child. It is an ongoing process, as the parents need to be counseled periodically at various stages of their child's development.