Cerebral Palsy: Definition, Classification, and Etiology

1. Cerebral Palsy - I Dr ShyamSudhir Professor Dept of Paediatrics

2. Definition ‡ Cerebral palsy (CP) describes a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain. ‡ The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, perception, cognition, communication, and behaviour, by epilepsy, and by secondary musculoskeletal problems. Report Exec Committee April 2006, DMCN Definition

3. What is cerebral palsy ? • Cerebral Palsy defined as “a group of non progressive but often changing motor impairment syndromes secondary to lesions or anomalies of brain arising in early stages of its development

4. Introduction – Contd.. • The lesion is permanent and non progressive • It includes non progressive genetic diseases or congenital malformations • Excludes cerebral tumors, degenerative brain diseases, progressive multi system diseases • This does not mean that CP is static condition • Clinical pattern changes as brain maturation continues throughout childhood

5. What is not Cerebral Palsy? • Deterioration of acquired motor activity over a period of time. • Hypotonic child develops hypertonia over a period of time during evaluation is a case of C.P. Reverse is not a C.P. • Mild Motor dysfunction improves over a period of time is not a case of C.P.

6. Classification of CP

7. Gross motor functional classification system (GMFCS) Level Function I Ambulatory in all settings II Walks without aides but has limitations in community settings III Walk with aides (hand held mo) IV Mobility requires wheel chair or adult assist V Dependent for mobility, Remain fairly stable over time

8. Research classification of CP • Monoplegia – spastic, rigid, hypotonic, dyskinetic, ataxic • Hemiplegia – spastic, rigid, hypotonic, dyskinetic, ataxic • Diplegia – spastic, rigid/dystonic, ataxic/hypotonic • Triplegia – diplegia + hemiplegia • Tetraplegia • Both arms – ataxia, tremor, dystonia • Truncal – ataxia ( hypotonia ) • Simple developmental delay • Unclassifiable

9. Incidence of CP • Spastic CP - 75% • Ataxic CP - 15% • Choreoathetotic CP - 5% • Prevalence of CP varies from 1.5 to 2.5/1000 live births.

10. Incidence of Comorbid Conditions with CP • Mental retardation - 50-75% • Most often in quadriplegia & ataxia CP • Seizures - 25-33% • Often in spastic hemiplegia & quadriplegia, least in dyskinetic CP • GTCS, partial complex, status can occur • Hearing & speech defects - 15 – 20 % • Common in dyskinetic & quadriplegic CP • Ocular defects - 50 -70 % • Refractive errors, strabismus • Behavioral problems - 30 -50 % • Hyperkinetic behavior, stubbornness, aggressiveness , lack of attention

11. Etiology • Traditional concepts regarding etiology have been radically challenged • “Birth asphyxia", once felt to be implicated in the majority of cases, has a causative role in approximately 10-15% of cases only • Antenatal factors are recognized as having a predominant etiological role in the pathogenesis of CP at present

12. Etiology – Contd.. • Recent studies have shown that cerebral palsy often has prenatal antecedents including congenital malformations, vascular insults and maternal infection • Cerebral palsy is therefore better viewed asoccurring among fetuses, rather than among infants

13. Etiology – Contd..

14. Etiology In Association With Four Motor Syndromes

15. Pathogenesis INTRAUTERINE/PERIPARTUM ASPHYXIA FETAL ↓PO2 ↑ PCO2 PH BP INTRACELLULAR EDEMA CEREBRAL TISSUE PRESSURE FOCAL CBF GENERALISED BRAIN SWELLING ↑ ICP GENERALISED CBF BRAIN NECROSIS BRAIN SWELLING

16. PARTIAL ANOXIA BRAIN SWELLING VARIOUS DEGREES OF CEREBRAL CONVOLUTIONAL FLATTENING NECROSIS WITH TISSUE SOFTENING AND FRIABILITY TISSUE COLLAPSE, SHRIVELLING, TOUGHENING OF INVOLUTED CONVULSION NODULAR SCLEROTIC MICROGYRIA

17. Pathogenesis ‡ Inflammatory mediators (maternal illness, fetal or neonatal) „ Cause damage or affect brain development „ Main cause in term babies without perinatal hypoxia ‡ Preterm babies: Multiple risk factors „ Apnea, hyperbilirubinemia, hypoxia, metabolic Problems

18. Neuropathological Varieties • Selective neuronal necrosis • Status marmoratus • Parasagital cerebral injury • Periventricularleucomalacia(PVL) • Focal/multifocal ischemic brain necrosis

19. Neuropathological Correlates

20. Periventricular Leucomalacia (PVL) • Definition - Necrosis of white matter in a characteristic distribution - the white matter adjacent to external angles of lateral ventricles involving the centrum semiovale, optic and acoustic radiations.

21. PVL • Occurs commonly in premature infant • Postnatal survival after the asphyxial insult of more than few days needed • Evidence of cardiorespiratory disturbance present • Early form of PVL - perinataltelencephalicleukoencephalopathy (contains hypertrophic astrcytes and amphophilic globules)

22. PVL - Common sites • Level of occipital radiation at the trigone of lateral ventricle • Level of cerebral white matter around foramen of monro • Reason-these sites form the border zones between the penetrating branches of MCA and PCA/ACA

23. Histological picture of PVL • After acute hypoxic ischemic insult 6-12hrs - coagulation necrosis 24-48hrs - microglia infiltration, Hypertrophic astrocyte Proliferation, endothelial hyperplasia 5 days - foamy macrophages

24. Status Marmoratus • Lesion involves basal ganglia (caudate nucleus and putamen) • Least common type of neuropathological variety • Most common in terms • Features - neuronal loss, gliosis, hypermyelination

25. Parasagital Cerebral Injury • Lesion involves cerebral cortex (parasagital, superomedial aspect of cerebral convexities) ,subcortical white matter • Injury bilateral and symmetrical • Common in full term infant • Etiology - impaired cerebral perfusion/impaired vascular autoregulation

26. Focal/Multifocal Ischemic Brain Necrosis • Porencephaly - single unilateral cavity within cerebral hemisphere that may or may not communicate with lateral ventricle • Hydranencephaly - both hemispheres reduced to fluid filled sacs of CSF • Multicystic encephalomalacia - multiple cavitated foci with cerebral necrosis

27. Focal/Multifocal Ischemic Brain Necrosis – Contd.. • Factors that favors immature brain for dissolution 1) High water content 2) Relative paucity of myelinated fibres 3) Deficient astroglial response

28. Focal cerbrovascular insufficiency Presumptive Vascular mal development Embolus Thrombus Generalised circulatory insufficiency Intrauterine hypoxic ischemic event Neonatal hypoxic ischemic event Infarction - MCA territory PVL Focal/Multifocal Ischemic Brain Necrosis - Etiology

29. High Risk Mother

30. High Risk Infant

31. History in CP

32. Antenatal History • Booked Case • Number Antenatal Visits • Immunization with T.T. • Iron & Folic acid tablets (100) • H/o. Hyper emesis gravidarum • Fever with Rash – painful lymphadenopathy • Threatened abortion • DM, HT, Anemia • Cardiac diseases • H/o Drug intake • H/o Radiation Exposure

33. History of Complications in Antenatal Period • Haemorrhage • OBST. Labour • Rup. of uterus • Severe anemia • Eclampsia • Sepsis • Intrauterine Infections TORCH • Mat Malnutrition • Placental insufficiency

34. Duration of Labour Gravida 1st Stage 2nd Stage Primi 16 hours 1 hour Multi 8 Hours 30min Natal • Nature of Delivery – Natural / Forceps /LSCS / Vacuum Ext. • Breech / ABN Presentation • Place of Delivery Home / Institutional • Person Conducting Delivery Train/ Not Trained

35. Post Natal • Birth weight – SGA/ AGA / LGA • Prematurity – Term or preterm • Birth Asphyxia • Neonatal Convulsions • H/o. Jaundice / Kerniterus • H/o. Head injuries • Activity of child – Excessive cry / Lethargic • Respiratory Distress • Feeding Difficulties • Also occur Birth Injuries IV Infusion Hypoglycemia • Other factors associated increased risk of CP are:- Low APGAR Score Neonatal Seizures

36. Family History • To rule out degenerative disorders • Metabolic disorders • History similar illness in the family • Chromosomal abnormalities

37. Signs & Symptoms of CP

38. Excessive lethargy and irritability High pitched CRY Poor head control Decreased spontaneous activity Constant fisting beyond 2/12 Delayed Social Smile Persistence of Primitive reflexes beyond 6/12 Lack of auditory response Delayed development mile stones Development of Hand preference before 1 year Drags feet like – commando Sign / Toe walking Bunny Hopping “W” sitting Reduced head circumference Oculovisual problems :roving eyes,no visual followig,persistent squint Early Pointers to suggest late development of C.P

39. Mother Complaints During 1st 4 Months • Early stratle • Scissoring • Arching of Body • Inequality of Movements of Limbs • Cortical Fisting • Delayed milestones (4S) • Diff. in Toilet care • Diff. in carrying the child in mothers lap • Persistence of Tonic neck reflex

40. What are soft neurological signs? • Attention Deficit • Choreo – Athetoid movements • Minor movements like Head bobbing and foot tapping • Dysdiadochokinesia • Finger agnosia • Verbal dyspraxia • Strabismus

41. Head to Foot Exam Primitive reflexes Moro ATNR Rooting / Sucking Grasp - Plantar Palmer Inv Movements Cerebellar signs ANS - Bladder, Bowel Meningeal signs Neck stiffness Brudzinski sign Kernigs sign Signs of ICT Spine and cranium Exam of Fundus Exam of other systems General Examination

42. Neuro Cutaneous Markers • Café – au – lait spots • Neurobromas • Tubers, Nodules • Ash leaf patch • Shagreen patch • Hypo / Hyper pigmented patches • Facial naevus

43. Eye Changes In C.P • Nystagmus • Strabismus • Cataract - Rubella • Cortical Blindness • Myopia • Retrolental fibroplasia • Optic atrophy • Papilladema

44. Fundus changes in C.P • Chorioretinites with Microcephaly with HSM – CMV • Chorioretinites with Hydrocephalus – Toxoplasmosis • Salt and Pepper appearance – Rubella

45. Spastic group CP • Commonest type • Cortical pyramidal cells affected • Increased tone & DTR charecteristic • Clasp knife rigidity to passive movements • Types : all 4 limbs - Quadriplegia LL> UL - Diplegia UL>LL - Double hemiplegia ½ of body- Hemiplegia

46. Spastic quadriplegia • 2/3 of spastic form& most severe form • Cause :birth asphyxia/ birth injury • Associated features: mental retardation feeding difficulties seizures, constipation growth failure • Wind swept deformities (hip dislocation,pelvic tilt, scoliosis etc.,)

47. Diplegic CP • Lower limb > upper limb central paraplegia • Cause: preterm – periventricular leukomalacia • Dystonic phase 4 to 8 months dystonic athetosis & opisthotonus • Spastic phase

48. Diplegic CP - Features • Scisrroring of legs characteristic • Difficulty in applying diaper • Cammando crawl • Delayed walking, tip toe walking • GENETIC DIPLEGIA No dystonic phase Diplegia of PKU & Cystinosis • ATAXIC DIPLEGIA Initially very floppy spasticity Asso with hydrocephalus

49. Hemiplegic CP • 90% congenital ; right>left • Lesion: cerebral cortex level ,MCA territory • Newborn: asymmetric moro focal clonic seizures asymmetry of movements fisting on affected side hand preference < 1 year • Normal IQ : 60% ;seizures : 15- 45% • UL signs develop early due to cephalocaudal development

50. Athetotic CP • Lesion : basal ganglion& corpus striatum • Cause : kernicterus asphyxia in prematurity • Involuntary movements slow writhing, sometimes choreic absent in sleep • Newborn opthistonus & irritability early features • IQ : Normal; dysarthria simulate MR • Hearng is often impaired