1. Cerebral Palsy
Hanan El Shakankiry
Consultant Pediatric Neurologist

2. 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

3. ? 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

4. 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

5. 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

6. ? 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

7. 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…

8. 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)

9. 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

10. 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

11. 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

12. 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 to appr.70
Moderate mental retardation: IQ level to 50-55
Severe mental retardation: IQ level to 35-40
Profound mental retardation: IQ level below 20 or 25

13. 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/

14. 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”

15. 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

16. Physiotherapy & Orthosis
MANAGEMENT OF SPASTISITY
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Intrathecal Baclofen
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Orthopedic Surgery
Physiotherapy & Orthosis
Phenol