Cerebra palsy Management - Dr. Ramya -Pediatrics

Cerebra palsy Management - Dr. Ramya -Pediatrics

1. Cerebral Palsy

2. Overview Definition Pathogenesis Clinical features Associated problems Differential diagnosis Management Prognosis

3. Cerebral palsy (CP)

4. 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.

5. 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.

6. 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.

7. 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.

8. 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

9. 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

10. Clinical Classification of CP Based on motor involvement Spastic- hypertonicity with poor posture control Hypotonic – despite pyramidal features infants are hypotonic. D yskinetic / athetoid - abnormal involuntary movement/slow wormlike writhing Ataxic- wide-based gait Mixed-type - combination of spasticity and athetosis

11. Based on topography Diplegia Quadriplegia and hemiplegia Accordingly , types include Spastic diplegia Spastic hemiplegia and Spastic quadriplegia

12. Clinical Classification of CP Based on motor involvement Spastic- hypertonicity with poor posture control Hypotonic – despite pyramidal features infants are hypotonic. D yskinetic / athetoid - abnormal involuntary movement/slow wormlike writhing Ataxic- wide-based gait Mixed-type - combination of spasticity and athetosis

13. Pathogenesis Selective neuronal Necrosis Parasagittal brain injury Status marmatosus Cerebellar nuclei involvement Periventricular leucomalacia

14. 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

15. Spastic diplegia Both lower limbs are more involved than upper limbs Intelligence usually preserved. Scissoring of lower limbs Tip toe walking

16. Spastic Quadripegia Severe form Cortical thumb Ophisthotonic posture Pseudo bulbar palsy Mental retardation Difficulty diapering due to spastic hip adductor muscles and lower extremities

17. 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

18. Dyskinetic Have extrapyramidal movements which interfere the normal movements Hearing is involved Not severely mentally retarded

19. Ataxic Least common type Ataxia due to cerebellar involvement

20. 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.

21. 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 defects ADHD- (may occur)-poor attention span, marked distractibility, hyperactive behavior

22. ASSOCIATED DISABILITIES Seizures - generalized tonic-clonic;more in postnatally acquired hemiplegia Drooling - may occur and lead to wet clothing/skin irritation Feeding - 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.

23. 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

24. 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

25. 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

26. 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).

27. 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

28. 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.

29. Differential diagnosis Neurodegenerative disorders Hydrocephalus and subdural effusions Brain tumors or ICSOL Muscle disorders Ataxia telangectasia

30. 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).

31. 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.

32. 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

33. 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

34. Surgical Intervention Reserved for child who does not respond to conservative therapy! Or whose spasticity causes progressive deformities Orthopedic surgery correct contractures or spastic deformities provide stability for uncontrolled joint provide balanced muscle power

35. 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.

36. 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

37. 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).

38. Service Coordination Case Management! Important for collaboration of all health professionals, services, therapies! Child needs support! Family needs support!