Minggu, 07 Oktober 2007

Cerebral Palsy

Definition

  • Disorder of movement and posturing

  • Caused by static brain lesion

  • Acquired during the stage of rapid brain development

  • Manifestations may change with growth and development

Epidemiology

Occurs in 1-5 in 1 000 live births. More common in advanced countries. Advanced perinatal care increases survival of brain-damaged children. Care only slightly reduces incidence of cerebral palsy . More common in socioeconomically disadvantaged.

Aetiology Aetiology

  • Prenatal (30%)

    • Maternal infection - Toxoplasmosis . Rubella . Cytomegalovirus . Herpes . Syphilis

    • Maternal exposure - Alcohol . Drugs

    • Congenital brain malformations

  • Perinatal

    • Birth weight <2500g>(25-40%)

    • Anoxia (10-20%)

  • Postnatal (10%)

    • Meningitis

    • Head injury

    • Immersion


Classification

  • 1. Spastic

    • Most common (60% of cases)

    • Most amenable to surgery

    • Due to upper motor neuron involvement - mild to severe motor impairment

    • Characterized by increased muscle tone and hyperreflexia, with slow, restricted movements (because of co contraction of agonist and antagonists)

    • Spasticity is characterized by increased muscle activity with increasingly rapid stretch (clasp knife & clonus)

    • Contractures

    • hemiplegia (both limbs on one side): arm usually worse than leg - all hemiplegics will walk, regardless of treatment; present with toe walking only

    • diplegia : have more extensive involvement of the lower extremity than the upper extremity; most diplegics will eventually walk; IQ may be normal, strabismus is common; gait is typically characterized by a crouched gait, toe walking, and flexed knees; heel cord lengthening alone may exacerbate crouched gait;

    • paraplegia (both legs): sparing of arms

    • quadriplegia : look for oral, lingual, dys f(x); dysarthria;

  • 2. Athetoid / Dyskinetic

    • Writhing movements. When excited, wriggle as if tickled.

    • 20% of cases

    • Result from basal ganglia involvement

    • Present w/ slow, writhing, involuntary movements

    • may affect the extremities (athetoid), or the proximal parts of limbs and the trunk (dystonic)

    • Hyperextended hips & knees with exaggerated stepping gait. Lean backwards, extending shoulder girdle & trunk.

    • Abrupt, jerky distal movements (Choreiform) also may occur;

    • Movements incr during with emotional tension and disappear during sleep.

    • Dysarthia is present and is often severe.

    • Intelligence normal (often above average)

    • Most difficult to correct with surgery - results are unpredictable & plaster immobilisation hazardous due to friction from constant movements.

  • 3. Ataxic

    • 10% of cases

    • Involvement of the cerebellum or its pathways

    • Weakness, inco-ordination, and intention tremor produce unsteadiness, wide based gait, and difficulty with rapid or fine movements

    • Poorly amenable to surgical correction;

  • 4. Hemiballistic

    • Sudden movements . As if throwing ball.

  • 5. Hypotonic

    • Usually a stage through which an infant passes.

  • 6. Combination


Pathogenesis

1. Weakness

Upper motor neuron lesion causes

  • Loss of voluntary movement

  • Weakness

  • Easy fatigability

2. Spasticity

  • Feature of all lesions of pyramidal system . Cerebral, capsular, pontine, midbrain lesions

  • Related to excessive activity of disinhibited spinal neurones

  • Mediated via stretch reflex . Muscle spindles detect stretch and stimulate muscle to contract . Threshold regulated by descending tracts . Spasticity due to hyperactivity of stretch reflexes

  • Tendon reflexes hypertonic . Clonus may appear

  • Posture characteristic because some neurones more active than others

  • Attempts to change position lead to resistance which quickly yields Clasp-knife phenomenon.

3. Contracture.

  • Nature of muscle contracture is: Shortening of muscle-tendon unit due to failure to keep pace with growth of bones.

  • Muscle adds sarcomeres at musculotendinous junction in response to constant stretch

  • In normal children, walking and movement provide all the stretch needed. When muscles spastic, this mechanism cannot occur.

4. Deformity

  • From unopposed muscle contracture.

  • Hip dislocation. Persistent hip adduction leads to valgus of femoral neck. Persistent hip flexion leads to anteversion of femoral neck. Results in Acetabular dysplasia, Hip subluxation & Hip dislocation

Note:

  • Spasticity - Abnormally increased contraction of a muscle in response to a stretch. Growth of muscles is impaired.

  • Rigidity - Involuntary sustained contraction of a muscle not stretch-dependent. Growth of muscles is not impaired.


Clinical features

1. Spastic quadriplegia. (25%)

  • Initially child floppy and will not feed

  • Choking during feeding from pseudobulbar palsy (difficult swallowing & chewing; dribbling)

  • Fail to thrive

  • Intelligence, vision and hearing affected

  • Only 10-20% will walk

  • Begin to walk up to age 7

  • Usually mentally retarded

  • Develop hip dislocation early and scoliosis.

2. Spastic diplegia.(30%)

  • All developmental milestones delayed

  • Most walk by age 4 .

3. Spastic hemiplegia (40%)

  • Usually noticed at walking age

  • Mean age of walking is 2-3 months later than normal.

  • Limp and one-handedness noted

  • Right-sided form may have speech delay

  • Seizures common.

  • Mild learning problems

  • Hyperactivity

4. Monoplegia (5%)

Associated disorders

  • Most common with total involvement

  • Mental retardation

  • Seizures

  • Learning disorders

  • Emotional and personality derangement

  • Visual defects

  • Hearing impairments

  • Disorders of speech


Assessment

History

  • Abnormal birth history

  • Prematurity

  • Neonatal nursery

  • Normal Developmental milestones (brackets are 95th percentile)

    • Head control -3 mths (6 mths)

    • Sitting independently - 6 mths (9 mths)

    • Crawling - 8 mths (never)

    • Pulling to stand - 9 mths (12 mths)

    • Walking -12 mths (18 mths)

Examination. (Also see CP Examination )

  • Walking- Arm swing . Trunk leans forward. Scissoring (d.t. Hip flexion & adduction). Windswept posture . Knee flexion . Stride length reduced . Narrow walking base. Equinus. Lordosis . Co-ordination in turning. [ Gait Analysis ]

  • Sitting - Legs forward or W . Upright or slouched.

  • Kneeling eliminates contracture effect .

  • Hips - Clinical signs of dislocation:

    1. Limited abduction, esp. with rapid stretch (grab test)

    2. Asymmetric knee height with pelvis level & knees flexed ( Galeazzi test )

    3. Windswept posture - one hip adducted & other side abducted

    4. Asymmetric leg length

    5. Hip flexion contractures

  • Muscles:

    • Psoas

      • FFD of hip demonstrated by Thomas test

      • Increased lumbar lordosis and prominent bottom when standing

      • Decreased sacrofemoral angle on standing lateral x-ray

      • Reduced SLR because of flexed pelvis from FFD.

    • Hamstrings

      • Reduced SLR

      • Hip extension contracture

      • FFD at knee

      • Lumbar kyphosis and small bottom when standing

      • Knee flexed at beginning of stance phase

      • False equinus (flexed knee lifts heel from ground)

      • Internal femoral torsion (sitting in W position)

      • Inability to touch toes

      • Reduced popliteal angle (hip at 90o)

      • High patella (flexed knee and spastic quadriceps)

    • Adductors

      • Scissored gait if bilateral

      • Apparent leg length discrepancy if unilateral

      • Trendelenberg limp

      • Decreased hip abduction

      • Eventual hip dislocation

    • Quadriceps

      • Stiff-legged gait (knees never flex)

      • Inability to flex knee when hip extended means rectus is responsible

      • Ely test (child prone, flex knee, if hip flexes = rectus femoris tight).

    • Triceps surae

      • Ankle equinus

      • Tiptoe gait

      • Recurvatum at knee when heel goes down

      • Silverskold's Test - equinus improves with knee flexion = soleus tighter than gastrocnemius

  • Neurology

    • Gross. Weakness

    • Clasp-knife phenomenon

    • Primitive reflexes

      • Moro reflex - Hold child at 45 o . Allow head to drop back . Arms and legs stick out in extension . Normally disappears by 4 mths.

      • Labyrinthine reflex - Tone reduced and arms and legs flex when child prone . Tone increased and arms and legs extend when child supine . Normally disappears by 6 mths.

      • Parachute reflex - When child held head down, both hands put out protectively . Appears at 5 mths.

  • Upper limbs

    • General.

    • Look at resting position.

    • Look for contractures.

    • Assess joint stability

    • Hand placement. Ask patient to place hand on knee and then head.

    • Control.

      • Ask patient to pretend to play piano . Look for independent movement.

      • Ask patient to throw object. Look for grasp and release.

      • Stereognosis. Test ability to recognise shape in palm

  • Predictors of Walking (from 1 year of age):

    1. Asymmetrical tonic neck reflex

    2. Symmetrical tonic neck reflex

    3. Neck righting reflex

    4. Moro reflex

    5. A pattern of extensor thrust & abduction of the legs when supported upright

    6. Parachute reflex

    7. Stepping reflex

    • If any 2 of these 7 responses are inappropriate by 1 year of age it is highly unlikely that the child will walk independently

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