CEREBRAL PALSY

Dr. ASIM SAEED BUTT

Define

Is defined as a :

1) Persistent but not unchanging

2) Disorder of movement, tone and posture

3) Due to non-progressive defect/lesion

4) Of 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 –

I. Mental retardation (60%)

II. Epilepsy (33%)

III. Visual , hearing (deafness-10%) and speech defects

IV. Strabismus(50%)

V. Cognitive dysfunction

VI. Sensory problems

VII. Emotional and behavioral problems.

CLASSIFICATIONS

TOPOGRAPHIC• MONO• HEMI• DIPLEGIA• QUADRI

• TRIPLEGIA

PHYSIOLOGY• SPASTIC• EXTRAPYRAMIDAL• ATAXIC• MIXED• ATONIC

FUNCTIONAL

• CLASS 1 – NO limitation of activity

• CLASS 2 – Slight limitation

• CLASS 3 – Moderate limitation

• CLASS 4 – No useful physical activity

Site of brain injury Pathological

• Periventricular leucomalacia –spastic diplegia

• Stroke in utero - hemiplegia• Multifocal encephalomalacia

-quadriplegia• Cerebellar - ataxic• Basal ganglia, thalmus,

putamen - dyskinetic

Etiological

Prenatal• I, iron def.,poor –nut.• Inf, UTI, high fever• Chorioamniotis• HTN, DM• Teratogens• Poor ANC• LOW SES• Twins• Fetal vasculopathy

Perinatal• Birth asphyxia• Premature / LBW• IUGR• Hyperbilirubenemia• IVH• Sepsis, pneumonia,

meningitis• Develop.

malformation

Postnatal•CNS infections•Head injuries•Seizures•Hypoxic damage•Hyperpyrexia damage

Early markers of CP

• SLOW head growth• Poor head control• Eye – roving eyes, poor hand

regard, persistent squint.• Ear – lack of auditory

response• Irritability, seizures, poor

suck, poor quality of sleep.• Extreme sensitivity to light

• Cortical thumb beyond 8 weeks

• Handedness before 2 yrs• Paucity of limb movements• Scissoring of lower limbs• Toe walking• Abnormal tone• Persistence of primitive

reflexes or failure to acquire postural reflexes

• Stereotypic abnormal movements

• Lack of alertness

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 circum ference 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,steping refex,tonic neck reflex .

• 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 Behaviour

• Mental 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 Feeding and Nutrition

• Oromotor 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 .

Assessment of Speech and Language

• These may be due to hearing impairment, cognitive deficits, or oromotor dysfunction

•

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

Orthopedic Problems

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

• Reduced bone density and propensity to fractures with 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 Neuroimaging• Neuroimaging (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 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.

• 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

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.

• 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 Therapy

• The 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.

DIFFERENT DEVICES

• 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. (

Pharmacological management

• 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.

Pharmacological management

• Baclofen has also been used intrathecally using implantable infusion pumps.

• It may be helpful in cases of severe spasticity or disabling total body dystonia

Pharmacological management

• 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.

Surgical management

• 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 .

counselling

• 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.

THANKS

THANKS