Disorders characterized by early onset and impaired movement and posture. It is a non-progressive

disease.

 1.9-2.3 in every 1000 live births   in prevalence since the 1960’s due largely to the improved survival of VLBW infants.

 Greatest prevalence is seen in prematurely delivered infants.

 Formerly thought to be R/T perinatal birth asphyxia, but now it is known that CP more commonly results from existing prenatal brain abnormalities.

 24% of cases have no identifiable cause.

 Difficult to determine exactly  ANOXIA is most significant factor to cause pathologic brain damage. This is often 2 ndary to other etiology.

 The area of the lesion in the brain mostly determines the subsequent pathology.

Spastic

: MOST COMMON

•

usually caused by hypoxic infarction in the area adjacent to the lateral ventricles.

•

Upper motor neuron type of muscular weakness.

•

See Box 40-3 (p.1693) for Types of Spastic Cerebral Palsy

•May involve one or both sides

.

•Hypertonicity with poor control of posture, balance, & coordinated motion •Impairment of fine and gross motor skill •Active attempts at motion increase abnormal postures and overflow of movement to other parts of the body

Dyskinetic/ athetoid —

•

Abnormal involuntary movement

•

Caused by kernicterus & hemolytic disease of the newborn which leads to pigment deposits in the basal ganglia & some cranial nerve nuclei .

•

Athetosis —

slow, wormlike writhing movements that usually involve the extremities, trunk neck, facial muscles, and tongue •Involvement of the pharyngeal, laryngeal, and oral muscles causes drooling and dysarthria (imperfect speech articulation) •Involuntary movements may take on choreoid (involuntary, irregular, jerking movements) and dystonic (disordered muscle tone) manifestations that increase in intensity with emotional stress and around adolescence.

Ataxic Mixed Type/ dystonic

•Wide-based gait •Rapid, repetitive movements performed poorly •Disintegration of movements of the upper of the upper extremities when the child reaches for objects

•Combination of spasticity and athetosis

 Delayed gross motor development— universal manifestation of CP  Especially significant if other developmental behaviors e.g. speech & personal social are normal  Abnormal motor performance— Early sign is preferential unilateral hand use that may be apparent at ~6months of age.

May stand or walk on toes

 Alterations of Muscle Tone—    or  resistance to passive movements is a sign of abnormal muscle tone.

Child may exhibit opisthotonic postures and stiffness on handling, dressing, or diapering.

 Abnormal Postures—   From an early age, a child lying in a prone position will maintain the hips higher than the trunk with the legs and arms flexed or drawn under the body.

Spasticity may be mild or severe.

  Reflex abnormalities—  Persistence of primitive infantile reflexes is one of the earliest clues to CP.

Associated Disabilities and problems—  Intellectual impairment—possible, but 70% are WNL    ADHD—poor attention span, marked distractibility, hyperactive behavior,and defects of integration Seizures—most common in postnatal acquired hemiplegia Drooling, feeding and speech needs,  risk of aspiration & possible inadequate gas exchange.

    Orthopedic complications Constipation Dental caries, malocclusion, gingivitis Nystagmus, amblyopia & hearing loss

 Neurologic Examination & History are the primary modalities for diagnosis  Recognizing etiologic factors that put the infant at risk is critical in the assessment and diagnostic process.

 Broad aims: 1.

Establish locomotion, communication, and self help 2.

3.

4.

5.

Gain optimum appearance & integration of motor functions Correct associated defects as effectively as possible Provide educational opportunities adapted to the needs and capabilities of the individual child Promote socialization experiences with other affected and unaffected children.

 Mobilizing devices—braces, crutches, wheelchairs, walkers  Surgery—when spasticity causes further deformities  Medication—drugs to  spasticity are often NOT helpful in CP. Antianxiety meds may help child with athetosis.Skeletal muscle relaxants e.g. baclofen, methocarbamol (Robaxim), or dantrolene (Dantrium) & Valium may help short-term for older children & adolescents. Antiepileptic meds, e.g. phenobarbital & phenytoin are used routinely for children with seizures & CP.

 Technical aids—e.g. electromechanical toys that use biofeedback; microcomputers combined with voice synthesizers, or activated with a head-stick, tongue,or other voluntary muscle movement over which the child has control.

 Other Considerations—care of vision & hearing deficits as well as dental care is essential early on.

 Physical Therapy—one of the most commonly used conservative tx modalites. Involves PT, family, and nsg  Functional & Adaptive Training (Occupational Therapy)—training in manual skills and ADL’s must be started early  Speech Therapy—start early to prevent speech problems.

 Education—individualize to the needs of the child  Recreation—sports, physical fitness, & recreation programs are encouraged for children with CP

   

Reinforce therapeutic plan/assist in Normalization Address Health Maintenance needs

 Watch for fatigue, nutritional needs, safety needs

Support family

 Help them cope with the emotional aspects of the disorder   Make appropriate referrals to support groups e.g United Cerebral Palsy Association. http://cerebralpalsy.org/ Support hospitalized child—  the nurse’s actions should convey acceptance, affection, and friendliness and promote a feeling of trust and dependability.

Gradual degeneration occurs in muscle fibers  progressive weakness and symmetric wasting away of skeletal muscle

 Pseudohypertophic (Duchenne)     X-linked Recessive 1-3 years of age Lordosis, Waddling gait Rapid progression—  Death 15-25 after onset   Website Part 1 with newest Guidelines from MDA—12/09 Part 2 with newest multidisciplinary guidelines from MDA—12 09: Bushby, K., Finkel, R., Birnkrant, D. J., Case, L. E., Clemens, P. R., Cripe, L., & ... Constantin, C. (2010). Diagnosis and management of Duchenne muscular dystrophy, part 2: implementation of multidisciplinary care. Lancet Neurology, 9(2), 177-189. doi:10.1016/S1474-4422(09)70271-6

 Onset after 8 y/o  Weakness of proximal muscles of pelvic and shoulder girdle  Slow progression    Incapacitated 20 years after onset OR slight disability

 Early adolescence  Symptoms    Lack of facial mobility Can’t raise arms over head Shoulders slope forward  VERY SLOW PROGRESSION

 Serum Creatinine Phosphokinase (CPK)  Electromyography (EMG)  Muscle Biopsy

 Supportive 

Physical Therapy



Orthopedic Trx (casting, bracing, surgery) to minimize deformities and maintain ability to perform ADL’s

 Most severe + most common type  X-linked recessive  Inherited MOTHER carrier/Son Symptoms  Genetic mutation—ABSENT skeletal muscle protein

 Muscle weakness by 3 y/o  Hx delayed motor development  Abnormal Gait, Waddling  Falls Frequently  Marked Lordosis when standing 

Gower’s Sign

 Enlarged calves, upper arms, thighs  fatty infiltration of muscle  pseudohypertrophic  Contractures  12 y/o = unable to walk  Weakened respiratory muscles  Death

 Contracture Deformities  Atrophy  Trx     PROM & AROM Casting/Bracing Rigid Corset Frequent Rest  PT  3 hrs of ambulaton/day

 Infections d/t decreased vital capacity and atrophy of resp muscles  Antibiotics  Resp. Trx  Chest Physiotherapy  Obesity d/t overfeeding and decreased activity   Diet Recreation as tol.

 Maintain mobility as long as possible

 D/T Weakening of Cardiac Muscle  Treatment  Digoxin  Diuretics e.g furosemide

 Serum Enzymes    Creatinine Phosphokinase Aldoase Glutamic-oxaloacetic transaminase (SGOT)  Very high levels in 1 st 2 years of life  Levels decrease as muscle deteriorates  WNL when severe wasting and disability

 Muscle Biopsy    Degeneration of muscle fibers Fatty deposits Fibrosis  EMG  Diminished duration and amplitude of existing motor unit potentials

 Help maintain independence  Continual evaluation of capabilities  Home Assessment   Set-up w/c assessible?, wide doors?, etc Buying clothes  Respite Care  Family Involvement  Genetic Counseling