Hanan El Shakankiry Hanan El Shakankiry Consultant Pediatric Neurologist Consultant Pediatric Neurologist Cerebral Palsy.

Hanan El ShakankiryHanan El Shakankiry Consultant Pediatric NeurologistConsultant Pediatric Neurologist

Cerebral Palsy

Definition of C PDefinition of C P

A group of disorders of motion and posture caused by brain A group of disorders of motion and posture caused by brain insult or injury occurring in the period of early cerebral insult or injury occurring in the period of early cerebral development (< 2yrs)development (< 2yrs)

Intellectual, sensory and behavioral difficultiesIntellectual, sensory and behavioral difficulties±±

It is a static encephalopathy; the result of It is a static encephalopathy; the result of brain dysfunction is neither episodic nor brain dysfunction is neither episodic nor

progressive progressive

However, the tone and postural However, the tone and postural abnormalities may become more pronounced abnormalities may become more pronounced during early childhood during early childhood && The full extent of motor disability may The full extent of motor disability may not not be evident until 3-4 yrs of agebe evident until 3-4 yrs of age

EtiologyEtiologyPrenatalPrenatal

Congenital brain defect Congenital brain defect Congenital anomalies external to CNSCongenital anomalies external to CNSIntrauterine infectionIntrauterine infectionHemolytic disease of the newbornHemolytic disease of the newbornFetal anoxiaFetal anoxiaTwinsTwinsMaternal diseasesMaternal diseases

NatalNatalPrematurityPrematurityTraumaTraumaHypoxic-ischemic encephalopathyHypoxic-ischemic encephalopathyInfectionInfection

PostnatalPostnatalHyperbilirubinemiaHyperbilirubinemiaHypoxia / AcidosisHypoxia / AcidosisIntracranial HgeIntracranial HgeInfectionInfectionMetabolicMetabolic

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Clinical PictureClinical Picture

Delay in milestones of developmentDelay in milestones of development

No loss of previously acquired skillsNo loss of previously acquired skills

+/- Persistence of neonatal reflexes: +/- Persistence of neonatal reflexes:

Presence of signs of UMNL Presence of signs of UMNL

Moro, Palmer & Planter grasp,Moro, Palmer & Planter grasp,Tonic neck, Rooting, SucklingTonic neck, Rooting, Suckling

Failure or delay of development of maturational reflexesFailure or delay of development of maturational reflexes

Classification of Cerebral PalsyClassification of Cerebral Palsy

Spastic CPSpastic CP

Dyskinetic CPDyskinetic CP

Ataxic Ataxic CPCP

Atonic CPAtonic CP

MixedMixed

Diplegic 44%Diplegic 44%HemiplegicHemiplegic 33% 33%Quadriplegic 6%Quadriplegic 6%Double Hemiplegic Double Hemiplegic MonoplegicMonoplegicParaplegicParaplegic

80%- 8580%- 85

Hypotonia + Normal or Increased ReflexesHypotonia + Normal or Increased Reflexes

Mainly AthetoidMainly AthetoidMainly DystonicMainly Dystonic

Hypotonia Cerebellar SignsHypotonia Cerebellar Signs

10-15%

TypeType

CauseCause

Associated with Associated with eg. eg. MicrocephalyMicrocephaly

MRMRSeizurSeizureses DeafnessDeafness

BlindnessBlindness

Complicated by Complicated by ContracturesContractures

GORDGORDMalnutritionMalnutrition

Chest infectionChest infection

eg. Post anoxiceg. Post anoxic

eg. Spastic quadriplegiceg. Spastic quadriplegic

Aggressive behaviorAggressive behavior

??

Assessment of the data gathered in the National Collaborative Prenatal Assessment of the data gathered in the National Collaborative Prenatal Project has tended to support Freud’s observations:Project has tended to support Freud’s observations:

In the original description of “cerebral palsy” by Little (1862) motor In the original description of “cerebral palsy” by Little (1862) motor dysfunction was attributed to intrapartum asphyxia and/or trauma.dysfunction was attributed to intrapartum asphyxia and/or trauma.

Freud, however, in 1897, proposed that abnormalities during labor and Freud, however, in 1897, proposed that abnormalities during labor and delivery were the result of an abnormal fetus entering the delivery delivery were the result of an abnormal fetus entering the delivery process rather than the delivery being the causative of neurologic process rather than the delivery being the causative of neurologic disability. disability.

Difficulties & D D Difficulties & D D I - Blaming intrapartum asphyxia:I - Blaming intrapartum asphyxia:

Epidemiological studies suggest that in about Epidemiological studies suggest that in about 90%90% of cases of cases intrapartum hypoxia could not be the cause of cerebral palsy and that intrapartum hypoxia could not be the cause of cerebral palsy and that in the remaining in the remaining 10%10% intrapartum signs compatible with damaging intrapartum signs compatible with damaging hypoxia may have had antenatal origin eg. Prematurity, intrauterine hypoxia may have had antenatal origin eg. Prematurity, intrauterine infection, antepartum haemorrhage, breech presentation, and infection, antepartum haemorrhage, breech presentation, and chromosomal or congenital anomalies…chromosomal or congenital anomalies…

Criteria to define an acute intrapartum hypoxic Criteria to define an acute intrapartum hypoxic eventevent

Essential criteriaEssential criteria

11 Evidence of a metabolic acidosis in intrapartum fetal, umbilical arterial cord, or 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) very early neonatal blood samples (pH <7.00 & base deficit 12 mmol/l)

22 Early onset of severe or moderate neonatal encephalopathy in infants of 34 Early onset of severe or moderate neonatal encephalopathy in infants of 34 weeks' gestation weeks' gestation

33 Cerebral palsy of the spastic quadriplegic or dyskinetic type Cerebral palsy of the spastic quadriplegic or dyskinetic type

Criteria that together suggest an intrapartum timing but by themselves Criteria that together suggest an intrapartum timing but by themselves are non-specific are non-specific

44 A sentinel hypoxic event occurring immediately before or during labour A sentinel hypoxic event occurring immediately before or during labour 55 A sudden, rapid & sustained deterioration of the fetal heart rate pattern usually A sudden, rapid & sustained deterioration of the fetal heart rate pattern usually

after the hypoxic event, the pattern was previously normalafter the hypoxic event, the pattern was previously normal66 Apgar scores of 0-6 for longer than 5 minutes Apgar scores of 0-6 for longer than 5 minutes77 Early evidence of multisystem involvement Early evidence of multisystem involvement 88 Early imaging evidence of acute cerebral abnormality Early imaging evidence of acute cerebral abnormality

The American Academy of Pediatrics collaborated with The American College of The American Academy of Pediatrics collaborated with The American College of Obstetricians and Gynecologists (ACOG)Obstetricians and Gynecologists (ACOG)

Factors that suggest another cause:Factors that suggest another cause:

Umbilical arterial base deficit less than 12 mmol/l or pH greater than 7.00Umbilical arterial base deficit less than 12 mmol/l or pH greater than 7.00

Major or multiple congenital or metabolic abnormalitiesMajor or multiple congenital or metabolic abnormalities

Early imaging evidence of longstanding neurological abnormalities for Early imaging evidence of longstanding neurological abnormalities for example, ventriculomegaly, porencephaly, multicystic encephalomalacia example, ventriculomegaly, porencephaly, multicystic encephalomalacia

Signs of intrauterine growth restrictionSigns of intrauterine growth restriction

Microcephaly at birthMicrocephaly at birth

Congenital coagulation disorders in the child Congenital coagulation disorders in the child

Presence of other major antenatal risk factors for cerebral palsy Presence of other major antenatal risk factors for cerebral palsy Presence of major postnatal risk factors for cerebral palsy eg. Presence of major postnatal risk factors for cerebral palsy eg. postnatal encephalitis, prolonged hypotension, or hypoxia due to severe postnatal encephalitis, prolonged hypotension, or hypoxia due to severe respiratory disease respiratory disease

A sibling with cerebral palsy, especially of the same typeA sibling with cerebral palsy, especially of the same type

i) Metabolic Etiologies of Neonatal Encephalopathy i) Metabolic Etiologies of Neonatal Encephalopathy

II- Conditions misdiagnosed as cerebral palsy:II- Conditions misdiagnosed as cerebral palsy:

Many metabolic conditions mimic asphyxia during the neonatal period. Many metabolic conditions mimic asphyxia during the neonatal period.

Many slowly progressive encephalopathies with onset Many slowly progressive encephalopathies with onset before age 2, can be mistaken for CP or post before age 2, can be mistaken for CP or post encephalitic sequelae encephalitic sequelae

-Nearly every chromosomal disorder has at least one abnormal neurologic -Nearly every chromosomal disorder has at least one abnormal neurologic manifestation. manifestation.

iii – Mental retardation, Chromosomal & Genetic iii – Mental retardation, Chromosomal & Genetic DisordersDisorders

-There is high frequency of mental retardation, seizures and -There is high frequency of mental retardation, seizures and anomalies involving the central nervous system in these diseases. anomalies involving the central nervous system in these diseases. They can be verified by cytogenetic testing and molecular DNA They can be verified by cytogenetic testing and molecular DNA diagnostic techniques diagnostic techniques

-Patients with -Patients with Pervasive developmental disordersPervasive developmental disorders have deviant development. They have significant have deviant development. They have significant deficits in speech and language and social adaptive deficits in speech and language and social adaptive domains but no evident motor deficits, however co domains but no evident motor deficits, however co morbidly, patients with CP may have MR, autistic morbidly, patients with CP may have MR, autistic features +/ or hyperactivity features +/ or hyperactivity

DSM-IV DIAGNOSTIC CRITERIA FOR MENTAL RETARDATIONDSM-IV DIAGNOSTIC CRITERIA FOR MENTAL RETARDATION

The onset is before age 18 yearsThe onset is before age 18 years

Significantly subaverage intellectual functioning: an I.Q. of Significantly subaverage intellectual functioning: an I.Q. of approximately 70 or below on an individually administered I.Q testapproximately 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.

Degree of severity Degree of severity

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

High percentage of patients with cortical maldevelopment High percentage of patients with cortical maldevelopment presented an intrapartum history suggestive of birth asphyxia presented an intrapartum history suggestive of birth asphyxia

iv - Cortical malformationsiv - Cortical malformations

Eg.Eg.-Worster–Drought syndrome-Worster–Drought syndrome ( bilateral perisylvian or opercular syndrome) ( bilateral perisylvian or opercular syndrome) Pseudobulbar palsy that presents with sucking and swallowing difficulties, Pseudobulbar palsy that presents with sucking and swallowing difficulties, excessive salivation, dysarthria, and an exaggerated jaw jerk.excessive salivation, dysarthria, and an exaggerated jaw jerk.Mild spastic diplegia or tetraplegia, variable cognitive and behavioural Mild spastic diplegia or tetraplegia, variable cognitive and behavioural impairment, and epilepsy. impairment, and epilepsy.

- Schizencephaly

- Heterotopia (double cortex )

- Lissencephaly/

Erbs palsyErbs palsy Tethered cordTethered cord

v- Disorders of lower motor neurons & anatomic v- Disorders of lower motor neurons & anatomic abnormalitiesabnormalities

MuscularMuscular dystrophies dystrophies

A-Global developmental delay in children with malnutritionA-Global developmental delay in children with malnutrition

B-Normal variationsB-Normal variations

““Bottom shuffling or scooting”Bottom shuffling or scooting”

One third of babies never crawlOne third of babies never crawl““just stand up”just stand up”

vi- Conditions over diagnosed as cerebral palsy:vi- Conditions over diagnosed as cerebral palsy:

ASSESSMENT AND EVALUATIONASSESSMENT AND EVALUATION

Team approachTeam approach

Pediatric neurologist Pediatric neurologist Physical therapistPhysical therapistOccupational therapistOccupational therapistSpeech therapistSpeech therapistPsychologistPsychologistSocial workerSocial workerNeurosurgeonNeurosurgeonOrthopedic surgeonOrthopedic surgeon

Multidisciplinary settingMultidisciplinary setting

IQIQ AudiometryAudiometry

Developmental assessmentDevelopmental assessment

Nutritional assessmentNutritional assessment

EEG if + seizuresEEG if + seizures

Access for Contractures, ScoliosisAccess for Contractures, Scoliosis

Eye & Fundus examinationEye & Fundus examination

TORCH screen Intrauterine infection suspectedTORCH screen Intrauterine infection suspected +/- R/O DD : eg.+/- R/O DD : eg.Chromosomal analysisChromosomal analysisMetabolic screeningMetabolic screening

Rehabilitation ProgramRehabilitation Program

Intrathecal Baclofen

Orthopedic Surgery

Orthopedic Surgery

MANAGEMENT OF SPASTISITYMANAGEMENT OF SPASTISITY

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Phenol

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Physiotherapy Physiotherapy & Orthosis& Orthosis