Cerebral Palsy Hanan El Shakankiry Consultant Pediatric Neurologist

Definition of C P A group of disorders of motion and posture caused by brain insult or injury occurring in the period of early cerebral development (< 2yrs) ± Intellectual, sensory and behavioral difficulties It is a static encephalopathy; the result of brain dysfunction is neither episodic nor progressive However, the tone and postural abnormalities may become more pronounced during early childhood & The full extent of motor disability may not be evident until 3-4 yrs of age

? Etiology Prenatal Natal Postnatal Congenital brain defect Congenital anomalies external to CNS Intrauterine infection Hemolytic disease of the newborn Fetal anoxia Twins Maternal diseases Natal Prematurity Trauma Hypoxic-ischemic encephalopathy Infection Postnatal ? Hyperbilirubinemia Hypoxia / Acidosis Intracranial Hge Infection Metabolic

Clinical Picture Delay in milestones of development No loss of previously acquired skills +/- Persistence of neonatal reflexes: Moro, Palmer & Planter grasp, Tonic neck, Rooting, Suckling Failure or delay of development of maturational reflexes Presence of signs of UMNL

Classification of Cerebral Palsy Spastic CP Diplegic 44% Hemiplegic 33% Quadriplegic 6% Double Hemiplegic Monoplegic Paraplegic 80%- 85 Dyskinetic CP Mainly Athetoid Mainly Dystonic 10-15% Atonic CP Hypotonia + Normal or Increased Reflexes Ataxic CP Hypotonia Cerebellar Signs Mixed

? Cause Type Associated with Complicated by eg. Post anoxic eg. Spastic quadriplegic Associated with eg. Microcephaly Seizures Aggressive behavior MR Deafness Blindness Complicated by GORD Contractures Malnutrition Chest infection

Difficulties & D D I - Blaming intrapartum asphyxia: In the original description of “cerebral palsy” by Little (1862) motor dysfunction was attributed to intrapartum asphyxia and/or trauma. Freud, however, in 1897, proposed that abnormalities during labor and delivery were the result of an abnormal fetus entering the delivery process rather than the delivery being the causative of neurologic disability. Assessment of the data gathered in the National Collaborative Prenatal Project has tended to support Freud’s observations: Epidemiological studies suggest that in about 90% of cases intrapartum hypoxia could not be the cause of cerebral palsy and that in the remaining 10% intrapartum signs compatible with damaging hypoxia may have had antenatal origin eg. Prematurity, intrauterine infection, antepartum haemorrhage, breech presentation, and chromosomal or congenital anomalies…

Criteria to define an acute intrapartum hypoxic event Essential criteria 1 Evidence of a metabolic acidosis in intrapartum fetal, umbilical arterial cord, or very early neonatal blood samples (pH <7.00 & base deficit 12 mmol/l) 2 Early onset of severe or moderate neonatal encephalopathy in infants of 34 weeks' gestation 3 Cerebral palsy of the spastic quadriplegic or dyskinetic type Criteria that together suggest an intrapartum timing but by themselves are non-specific 4 A sentinel hypoxic event occurring immediately before or during labour 5 A sudden, rapid & sustained deterioration of the fetal heart rate pattern usually after the hypoxic event, the pattern was previously normal 6 Apgar scores of 0-6 for longer than 5 minutes 7 Early evidence of multisystem involvement 8 Early imaging evidence of acute cerebral abnormality The American Academy of Pediatrics collaborated with The American College of Obstetricians and Gynecologists (ACOG)

Factors that suggest another cause: Umbilical arterial base deficit less than 12 mmol/l or pH greater than 7.00 Major or multiple congenital or metabolic abnormalities Early imaging evidence of longstanding neurological abnormalities for example, ventriculomegaly, porencephaly, multicystic encephalomalacia Signs of intrauterine growth restriction Microcephaly at birth Congenital coagulation disorders in the child Presence of other major antenatal risk factors for cerebral palsy Presence of major postnatal risk factors for cerebral palsy eg. postnatal encephalitis, prolonged hypotension, or hypoxia due to severe respiratory disease A sibling with cerebral palsy, especially of the same type

i) Metabolic Etiologies of Neonatal Encephalopathy II- Conditions misdiagnosed as cerebral palsy: i) Metabolic Etiologies of Neonatal Encephalopathy Many metabolic conditions mimic asphyxia during the neonatal period. Many slowly progressive encephalopathies with onset before age 2, can be mistaken for CP or post encephalitic sequelae

iii – Mental retardation, Chromosomal & Genetic Disorders -Nearly every chromosomal disorder has at least one abnormal neurologic manifestation. -There is high frequency of mental retardation, seizures and anomalies involving the central nervous system in these diseases. They can be verified by cytogenetic testing and molecular DNA diagnostic techniques -Patients with Pervasive developmental disorders have deviant development. They have significant deficits in speech and language and social adaptive domains but no evident motor deficits, however co morbidly, patients with CP may have MR, autistic features +/ or hyperactivity

DSM-IV DIAGNOSTIC CRITERIA FOR MENTAL RETARDATION Significantly subaverage intellectual functioning: an I.Q. of approximately 70 or below on an individually administered I.Q test Concurrent deficits or impairments in adaptive functioning in at least two of the following areas: communication, self-care, home living, social\interpersonal skills, use of community resources, self-direction, functional academic skills, work, leisure, health, and safety. The onset is before age 18 years Degree of severity Mild mental retardation: IQ level 50-55 to appr.70 Moderate mental retardation: IQ level 35-40 to 50-55 Severe mental retardation: IQ level 20-25 to 35-40 Profound mental retardation: IQ level below 20 or 25

iv - Cortical malformations High percentage of patients with cortical maldevelopment presented an intrapartum history suggestive of birth asphyxia Eg. -Worster–Drought syndrome ( bilateral perisylvian or opercular syndrome) Pseudobulbar palsy that presents with sucking and swallowing difficulties, excessive salivation, dysarthria, and an exaggerated jaw jerk. Mild spastic diplegia or tetraplegia, variable cognitive and behavioural impairment, and epilepsy. - Schizencephaly - Heterotopia (double cortex ) - Lissencephaly/

vi- Conditions over diagnosed as cerebral palsy: v- Disorders of lower motor neurons & anatomic abnormalities Erbs palsy Tethered cord Muscular dystrophies vi- Conditions over diagnosed as cerebral palsy: A-Global developmental delay in children with malnutrition B-Normal variations “Bottom shuffling or scooting” One third of babies never crawl “just stand up”

Team approach ASSESSMENT AND EVALUATION Multidisciplinary setting Developmental assessment TORCH screen Intrauterine infection suspected +/- R/O DD : eg. Chromosomal analysis Metabolic screening IQ Audiometry Nutritional assessment EEG if + seizures Eye & Fundus examination Access for Contractures, Scoliosis Team approach Pediatric neurologist Physical therapist Occupational therapist Speech therapist Psychologist Social worker Neurosurgeon Orthopedic surgeon Multidisciplinary setting Rehabilitation Program

Physiotherapy & Orthosis MANAGEMENT OF SPASTISITY *** Intrathecal Baclofen *** - - Orthopedic Surgery Physiotherapy & Orthosis Phenol