Sunday, October 28, 2007

Cerebral Palsy by Dr. Ameen Jan

CASE SCENERIO
•A 2 yr old child present with inability to sit, stand or walk. He is interactive and can speak few words. His weight is 10 Kg, head circumference 47 Cm, fontanel closed and other system examination unremarkable.
•His parents are young and noncosanguous. Pregnancy was full term and without complication. Delivery was at home and after prolonged and difficult labor. He has 4 other sibs and all normal.
Most likely Diagnosis
•Down syndrome
•Hypothyroidism
•Cerebral palsy
•Neurodegenerative disorder
•Leukodystrophy
•Congenital rubella syndrome


CEREBRAL PALSY
Dr. Ameen Jan
v The term CP include a variety of nondegenerative neurological disabilities caused by abnormal CNS development or injuries in the pre,peri or early postnatal period that result in abnormalities of motor function.OR
v CP is a disorder of posture, movement and tone due to a static encephalopathy ,acquired during brain growth in fetal life, infancy or early childhood.
v a nonprogressive disorder
v of posture and movement
v resulting from a defect or lesion of the developing brain -- acquired early in life
v The condition was first described >100yr ago by William Little an orthopedic surgeon
v It is a common disorder, estimated prevalence is 2-4 per 1,000 population.
v Associated with
v Epilepsy (35%)
v MR & abnormalities of speech, vision, intellect ( 30-70 5)
v The motor handicap may be the least of all problems.

Etiology
•Prenatal – infection - anoxia
- metabolic - toxic
- genetic - infarction
•Perinatal – anoxia -infection
•Postnatal – toxins - trauma
- infection
About 50% of CPs have no identifiable cause or risk factor
•low birth weight infants, particularly those weighing less than 1,000g at birth, are prone ,primarily because of intracerebral hemorrhage and periventricular leukomalacia.
•Birth asphyxia is an uncommon cause of CP
•Intrauterine exposure to maternal infection , umbilical cord inflammation, foul-smelling amniotic fluid, maternal sepsis, temperature greater than 38°C during labor, and urinary tract infection are associated.
•substantial number of children with CP had congenital anomalies external to the CNS

CLASSIFICATION

Physiologic classification identifies the major motor abnormality
•Spastic
•Athetoid
•Rigid
•AtaxicTremor
•Atonic
•Mixed
•Unclassified


CLASSIFICATION-Topographic
The topographic or Regional classification indicates the involved extremities.
•Monoplegia
•Paraplegia
•Hemiplegia
• Triplegia
• Quadriplegia
• Diplegia
• Double hemiplegia


CLASSIFICATION-Functional
•Class I — no limitation of activity
• Class II —slight to moderate limitation
• Class III —moderate to great limitation
•Class IV —no useful physical activity


Spastic hemiplegia
•have decreased spontaneous movements on the affected side and show hand preference at a very early age.
•The arm is often more involved than the leg
•Walking is usually delayed until 18–24 mo, and a circumductive gait is apparent.
---
•Extremities may show growth arrest, particularly in the hand and thumbnail
•Spasticity - at ankle - an equinovarus deformity of the foot.

•Spastic child often walks on tiptoes because of the increased tone

•Ankle clonus and a Babinski sign may be present
•Deep tendon reflexes are increased

•About 1/3ed of patients with spastic hemiplegia have seizure disorder and approximately 25% have cognitive abnormalities
Spastic quadriplegia
is the most severe form of CP because of marked motor impairment of all extremities and the high association with mental retardation and seizures.
• Neurological examination shows
-increased tone and spasticity in all extremities
-decreased spontaneous movements
- brisk reflexes, and plantar extensor responses.
•- Flexion contractures of the knees & elbows are often present by late childhood.
•-speech & visual abnormalities, are particularly prevalent in this group
•-Children with spastic quadriparesis often have evidence of athetosis and may be classified as having mixed CP.
Spastic diplegia
•is bilateral spasticity of the legs.
•Spastic diplegia is often first noted when an affected infant begins to crawl.The child uses the arms in a normal reciprocal fashion but tends to drag the legs behind more as a rudder (commando crawl) rather than using the normal four-limbed crawling movement.
•If the spasticity is severe, application of a diaper is difficult owing to excessive adduction of the hips.
•Walking is significantly delayed;


CLINICAL MANIFESTATIONS

Examination
•of the child reveals spasticity in the legs with brisk reflexes, ankle clonus, and a bilateral Babinski sign.
•When the child is suspended by the axillae, a scissoring posture of the lower extremities is maintained.
•Severe spastic diplegia is characterized by disuse atrophy and impaired growth of the lower extremities and by disproportionate growth with normal development of the upper torso.
•The prognosis for normal intellectual development is excellent for these patients, and the likelihood of seizures is minimal.
ATHETOID CP
•The athetoid movements may not become evident until 1yr of age and tend to coincide with hypermyelination of the basal ganglia,
•Speech is typically affected owing to involvement of the oropharyngeal muscles. Sentences are slurred,
• Generally, upper motor neuron signs are not present, -seizures are uncommon, and intellect is preserved in most patients.

DIAGNOSIS
•A thorough history and physical examination
•CT scan ,MRI may be indicated. A CT scan or MRI
may show an atrophic cerebral hemisphere ,infarction or thromboembolisim
•EEG
•D/D should include a progressive disorder of the CNS, including degenerative diseases, spinal cord tumor, or muscular dystrophy
•Additional studies may include tests of hearing and visual function.


Treatment
•A multidisciplinary approach is most helpful in the assessment and treatment of such children.
•A team of physicians from various specialties as well as the occupational and physical therapists, speech pathologist, social worker, educator, and developmental psychologist.
• Parents should be taught how to handle their child in daily activities such as feeding, carrying, dressing,
• adaptive equipment, such as walkers, poles, and standing frames may be helpful.
Surgery
•an adductor tenotomy or psoas transfer and release. A rhizotomy procedure in which the roots of the spinal nerves are divided has produced considerable improvement in selected patients with severe spastic diplegia.
•A tight heel cord in a child with spastic hemiplegia may be treated surgically by tenotomy of the Achilles tendon.
Drug Treatment
• Several drugs are used to treat spasticity, including dantrolene sodium, benzodiazepines, and baclofen.
•Patients with incapacitating athetosis may respond to levodopa,Ternaline etc
• children with dystonia may benefit from carbamazepine or trihexyphenidyl.

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