cerebra palsy management - dr. ramya -pediatrics
TRANSCRIPT
Cerebral Palsy
Overview
• Definition• Pathogenesis• Clinical features• Associated problems• Differential diagnosis• Management• Prognosis
Cerebral palsy (CP)
Definition
• It is a heterogeneous group of disorder with persistent disorder of movement and posture caused by non progressive defects or lesions of immature brain.
• Non-specific term• Non-progressive and may include perceptual
problems, language deficits, and intellectual involvement.
CP history
• Dr William John Little detected this disorder first in 1860.
• Sometimes known as Little’s disease.• Incidence has increased since the 1960 ’s,
maybe due to improved survival of VLBW infants.
Incidence
• Most common physical disability of childhood.• Incidence has increased since the 60’s, maybe
due to improved survival of VLBW infants.• Difficult to estimate accurate incidence.• Approximately 1-2 /100 live births in India.
Etiology• Variety of perinatal, prenatal, and postnatal factors
contribute, either singly or multifactorily to CP.• Commonly thought to be due to birth asphyxia; now
known to be due to existing prenatal brain abnormalities.
• Premature delivery is the single most important determinant of CP.
• In 24% of cases, no cause is found.
Causes of CP
• Time (% of cases)• Prenatal (44%)– First trimester
– Second trimester
• Causes
• Teratogens, chromosomal abnormalities, genetic syndromes, brain malformations
• Intrauterine infections, problems in fetal/placental functioning,coagulation & thrombosis
Causes of CP• Time (% of cases)• Labor and delivery (19%)
• Perinatal (8%)
• Childhood (5%)
• Not obvious (24%)
• Causes• Preeclampsia,
complications of labor and delivery, multiple births.
• Sepsis/CNS infection, asphyxia, prematurity, acid base imbalance, indirect hyperbilirubinemia,
• Meningitis, traumatic brain injury, toxins
Clinical Classification of CP
Based on motor involvement• Spastic-hypertonicity with poor posture control• Hypotonic – despite pyramidal features infants are
hypotonic.• Dyskinetic/athetoid- abnormal involuntary
movement/slow wormlike writhing • Ataxic- wide-based gait• Mixed-type- combination of spasticity and athetosis
• Based on topography• Diplegia• Quadriplegia and hemiplegia• Accordingly , types include• Spastic diplegia• Spastic hemiplegia and• Spastic quadriplegia
Clinical Classification of CP
Based on motor involvement• Spastic-hypertonicity with poor posture control• Hypotonic – despite pyramidal features infants are
hypotonic.• Dyskinetic/athetoid- abnormal involuntary
movement/slow wormlike writhing • Ataxic- wide-based gait• Mixed-type- combination of spasticity and athetosis
Pathogenesis
• Selective neuronal Necrosis• Parasagittal brain injury• Status marmatosus• Cerebellar nuclei involvement• Periventricular leucomalacia
Clinical manifestations
• Delayed gross motor development– A universal manifestation of CP– The discrepancy between motor ability and
expected achievement tends to increase as growth advances.
– Delayed development of ability to balance slows milestones
– Delay in all motor accomplishments
Spastic diplegia
• Both lower limbs are more involved than upper limbs
• Intelligence usually preserved.• Scissoring of lower limbs• Tip toe walking
Spastic Quadripegia
• Severe form• Cortical thumb• Ophisthotonic posture• Pseudo bulbar palsy• Mental retardation• Difficulty diapering due to spastic hip adductor
muscles and lower extremities
Spastic hemiplegia
• Usually detected only at 4-6 months of age due to hand preference
• Upper limb more effected than lower limb• Abnormal gait• Usually walk• Good mentation
Dyskinetic
• Have extrapyramidal movements which interfere the normal movements
• Hearing is involved• Not severely mentally retarded
Ataxic
• Least common type• Ataxia due to cerebellar involvement
Reflex Abnormalities
• Persistence of primitive infantile reflexes (one of the earliest signs of CP)– Tonic neck reflex– Hyperactivity or moro, plantar, palmar grasp.
Hyperreflexia, ankle clonus, stretch reflexes can be elicited from any muscle group.
Associated disabilities and problems
• Intellectual impairment– 70% w/in normal limits; wide range – Tests should be carried out over a period of time.– Children with athetosis and ataxia more intelligent.Speech difficulties (not a sign or MR)- child has motor and
sensory defectsADHD- (may occur)-poor attention span, marked
distractibility, hyperactive behavior
ASSOCIATED DISABILITIESSeizures- generalized tonic-clonic;more in postnatally
acquired hemiplegiaDrooling- may occur and lead to wet clothing/skin irritationFeeding- alterations in muscle tone lead to difficulties
chewing, swallowing, talking, etc.Address nutritional concerns.Coughing, choking may lead to aspiration.Altered respiratory patterns may lead to inadequate gas
exchange.
Associated Problems• Dental carries– Improper dental hygiene– congenital enamel defects (hyperplasia of primary teeth)– high carbohydrate intake and retention– Dietary balance with poor nutritional intake– Inadequate fluoride – Difficulty in mouth closure and drooling– Spastic or clonic movements cause gagging or biting on
toothbrush
Associated problems
• Nystagmus and amblyopia common– May need surgery or corrective lenses– May be due to sensoneural involvement– Infants lying flat too long may have otitis media
which may leads to conductive hearing loss
Diagnostic Studies
• Physical Assessment• Observe LBW, preterm, and those with low
Apgar scores at 5 minutes.• Observe infants who have seizures,
intracranial hemorrhage, metabolic disturbances
DX studies• Since control of movement does not occur until
late infancy, dx may not be confirmed until after 6 months of age.
• Diagnosis is mainly clinical with detailed history and clinical examination.
• IEM should be ruled out by urine and plasma aminoacids and reducing substances.
• MRI, eye examination, hearing assessment , karyotyping (if syndromic features are present).
WARNING SIGNS
• Physical Signs• poor head control after 3 months• stiff or rigid arms/legs, arching back, floppy or limp
posture• Cannot sit up without support by 8 months• Uses only one side of the body or only the arms to
crawl
Warning Signs
• Behavioral Signs• Extreme irritability or crying• Failure to smile by 3 months• Feeding difficulties– Persistent gagging or choking when fed– After 6 months of age, tongue pushes soft food
out of the mouth.
Differential diagnosis
• Neurodegenerative disorders• Hydrocephalus and subdural effusions• Brain tumors or ICSOL• Muscle disorders• Ataxia telangectasia
Therapeutic management• PHYSICAL THERAPY– Most commonly used treatments.– Goal is good skeletal alignment for the spastic child. – For the child with athetosis, training in purposeful acts,
even in the face of involuntary motion– Maximum development of proprioceptive sense for the
child with ataxia.– Orthotic devices (braces, splints, casting).
OCCUPATIONAL THERAPY
• Sitting to walking; feeding to cooking.• Important to incorporate play into program• Adaptive equipment (utensils for functional use, i.e.,
eating, writing), computers, etc.
Speech/Language therapy
• Early speech training by speech/language pathologist – Before child develops poor habits– Advice parents to follow directions of therapist– May need to force child to use tongue/lips in
eating
Special Education
• Determined by child’s needs• Early intervention programs• Individualized Education Program (IEP)• Specialized learning programs and support
services in schools• Socialization to promote self-concept
development
Surgical Intervention
• Reserved for child who does not respond to conservative therapy!– Or whose spasticity causes progressive deformitiesOrthopedic surgery– correct contractures or spastic deformities– provide stability for uncontrolled joint– provide balanced muscle power
Surgical Therapy
• Tendon-lengthening procedures (heel-cord)• Release of spastic wrist flexor muscles• Correction of hip-adductor muscle spasticity or
contracture to improve locomotion• Surgery is for improved function rather than cosmetic
reasons and is followed by PT.
Medication Therapy• Little usefulness • Anti-anxiety agents may relieve excessive motion and
tension (child with athetosis)– Skeletal muscle relaxants ,dantrolene (Dantrium), Baclofen,
may be used short-term for older children and adolescents.– Diazepam (Valium) for older children and adolescents, may
relieve stiffness and ease motion
Medications
• Local nerve blocks to motor points of a muscle with a neurolytic agent (phenol solution) may relieve spasticity.
• Botulism toxin (Botox) used to paralyze certain muscles.
• Pain• Secondary conditions (seizures, bowel and bladder
problems, lung complications).
Service Coordination
• Case Management!• Important for collaboration of all health
professionals, services, therapies!• Child needs support!• Family needs support!