SICU Meeting Cerebral Palsy

1. SICU MeetingCerebral Palsy ??:2003.06.30 ???:Ri???

2. Overview Cerebral palsy (CP) is a static encephalopathy caused by an insult to the brain during the prenatal, perinatal, or postnatal period CP is nonprogressive in nature. The clinical expression of CP is subject to change as children and their developing nervous systems mature. CP has been traditionally classified based on the type of motor disorder.

3. Definition Cerebral palsy is a symptom complex, rather than a specific disease. Definition: CP is an Umbrella term covering a group of non-progressive, but often changing, motor impairment syndromes secondary to lesions or anomalies of the brain arising in the early stages of its development Dev Med Child Neurol 1992;34:547-551

4. Classification Spastic, Comprises 70-80% of cases ?Further classified from extremity involvement Quadriplegia (10-15%) Diplegia (30-40%) Hemiplegia (20-30%) Monoplegia (rare) Dyskinetic, 10-15% of cases Ataxic, <5% of cases

5. Epidermiology Incidence:1.5-2.0 cases per 1000 live births Mortality/Morbidity: CP is the leading cause of childhood disability affecting function and development Age: Initial insult occurs between birth and age 2 years, but children usually are not diagnosed until after 1 year of age

6. Pathophysiology An insult to the immature brain at any time prior to birth up to 2 years of age Cerebral insult produces alterations in muscle tone muscle stretch reflexes primitive reflexes Secondary to the neurological insult mental retardation, vision and hearing problems, seizures

7. Clinical presentation (1) Failing to meet expected developmental milestones or failing to suppress obligatory primitive reflexes. Abnormal muscle tone:the most frequently symptom. An early period of hypotonia followed by hypertonia. Asymmetric crawl or failure to crawl

8. Clinical presentation(2) Persistent primitive reflexes,such as Moro reflex(4-6) Asymmetric tonic neck(6-7) Symmetric tonic neck(6-7) Palmar grasp(5-6) Tonic labyrinthine(4-6) Foot placement(12)

9. Primitive Reflex

10. Different Type of CP(1) Spastic hemiplegia One-sided upper motor neuron deficit Arm generally affected more than the leg; possible early hand preference or relative weakness on 1 side Oromotor dysfunction Specific learning disabilities Possible unilateral sensory deficits Visual field deficits (eg, homonymous hemianopsia) and strabismus Seizures cognitive impairment :28%

11. Different Type of CP(2) Spastic diplegia Upper motor neuron findings in the legs more than the arms Scissoring gait pattern with hips flexed and adducted, knees flexed with valgus and ankles in equinus (resulting in toe-walking) Related to Low birth body weight Learning disabilities and seizures less commonly than in spastic hemiplegia

12. Different Type of CP(3) Spastic quadriplegia than legs All limbs affected Oromotor dysfunction most patients with spastic quadriplegia h most patients with spastic quadriplegia have some cognitive impairment Multiple medical complications Seizures Legs generally affected equally or more than arm

13. Different Type of CP(4) Dyskinetia (extrapyramidal) Early hypotonia with movement disorder emerging when aged 1-3 years Arms more affected than legs Deep tendon reflexes usually normal to slightly increased Oromotor dysfunction Gait difficulties Truncal instability Risk of deafness in those affected by kernicterus Intelligence is normal in 78% of patients

14. Etiology Multifactorial but in most cases is unknown. Neuroimaging may be entirely normal. An increasing amount of literature suggests a link between various prenatal, perinatal, and postnatal factors and CP Prenatal factors play a significant role in the etiology of CP.

17. Prenatal Risk Factor Maternal thyroid disorder Long menstrual cycle Previous pregnancy loss Previous loss of newborn Maternal mental retardation Maternal seizure disorder History of delivering a child of less than 2000 g birth weight or with motor deficit, mental retardation, or sensory deficit

18. Factors during pregnancy Polyhydramnios Treatment of the mother with thyroid hormone Treatment of the mother with estrogen or progesterone A fetus with congenital malformation Maternal seizure disorder, severe proteinuria, or high blood pressure Bleeding in the third trimester Multiple gestations may not be an added risk for CP.

19. Perinatal Placentas show evidence of chorionitis were more likely than others to have CP. Chorionitis is thought to contribute either directly or indirectly by increasing the risk of prematurity. . Neonatal asphyxia

20. Postnatal Factors that lead to CP Infection such as meningitis encephalitis Intracranial hemorrhage hypoxia- ischemia from meconium aspiration Coagulopathies Trauma

21. Possible causes of CP by type Spastic hemiplegiae congenital and 70-90% are congenital and 10-30% acquired (eg, vascular, inflammatory, traumatic). In unilateral lesions of the brain, the vascular territory most commonly affected is the middle cerebral artery; the left side is twice as commonly involved as the right. Other structural brain abnormalities include hemibrain atrophy and posthemorrhagic porencephaly. In the premature infant, this may result from asymmetric periventricular leukomalacia.

22. Possible causes of CP by type Spastic diplegia In the premature infant, spastic diplegia may result from parenchymal-intraventricular hemorrhage or periventricular leukomalacia. In the term infant, no risk factors may be identifiable or the etiology might be multifactorial.

23. Possible causes of CP by type Spastic quadriplegia 50% of prenatal, 30% perinatal,20% postnatal in origin. This type is associated with cavities that communicate with the lateral ventricles, multiple cystic lesions in the white matter, diffuse cortical atrophy, and hydrocephalus. The patient often has a history of a difficult delivery with evidence of perinatal asphyxia. Preterm infants may have periventricular leukomalacia. Full-term infants may have structural brain abnormalities or cerebral hypoperfusion in a watershed

24. Possible causes of CP by type Dyskinetic (extrapyramidal) Associated with hyperbilirubinemia in the term infant or prematurity without prominent hyperbilirubinemia. Hypoxia affecting the basal ganglia and thalamus may affect the term infant more than the preterm infant.

26. workup Lab: Thyroid studies Lactate/ pyruvate level Organic and amino acids Ammonia Chromosomal analysis

27. workup Imaging study: No study exists to support definitive diagnosis of CP. Ultrasound Head CT MRI: spinal or brain EEG/EMG

30. Treatment Early Intervention Medical control Occupational therapy Physical Therapy Orthopaedic surgery Family education Neurosurgery :selective dorsal rhizotomy Orthosis, seating aids, walking aids

31. Medical Control Goal: reduce morbidity and prevent complication Seizure: Anticonvulsants Spasticity: Botulinum Toxin, Muscle relaxants Vision: Hearing: Bradykinesia,rigidity: Anticholinergics

32. Prognosis(1) Spastic quadriplegic patients 25% have minimal or no functional limitation in ambulation and self care 50% moderate impairment and are not independent but are able to function 25% impaired severely, require complete care, and are not ambulatory

33. Prognosis(2) Dyskinetic patients: 50% of patients are ambulatory. Hemiplegic patients: Most patients with CP become independent in activities of daily living (ADL) but may need assistive devices; most become ambulatory.

34. Prognosis(3) A prospective study of children suggested that being able to sit by the age of 2 years was a good predictive sign of eventual ambulation. Children who did not sit by the age of 4 years did not ambulate. Suppression of obligatory primitive reflex activity by 18-24 months provides a sensitive indicator to distinguish children who ultimately walked from those who would not be expected to walk.