Cerebral Palsy

Cerebral Palsy
HusSaM Al HaLaBi

 “An umbrella term that defines a group of non-progressive, but often changing, syndromes of motor impairment secondary to lesions or anomalies of the brain arising in the early stages of its development”
 Post neonatal events account for 12-21%

Classification
 …according to the extremities involved (monoplegia, hemiplegia, diplegia, and quadriplegia)
 …according to the characteristics of the neurological dysfunction (spastic, hypotonic, dystonic, and athetotic, or a combination)
Epidemiology
 Prevalence of moderately severe or severe CP is 1.5 to 2.5 per 1000
 Developed and underdeveloped countries have identical cerebral palsy rates
Clinical Patterns
 Full term infants:
 Hemiplegic CP is the commonest pattern
 Cerebral injury in the distribution of the middle cerebral artery is the most common finding in patients with hemiplegic spastic CP
 Some have periventricular atrophy, and some have malformations of cerebral development
 CT/MRI normal in ¼…some CP related to abnormalities at the microscopic level

 Premature infants
 Diplegic CP the commonest form
 Periventricular leukomalacia (PVL): foci of coagulative necrosis in the white matter near the lateral ventricles
 Susceptibility of fetal brain to PVL peaks at 28 weeks, and falls by 32 weeks
 these areas carry fibers responsible for the motor control and muscle tone of the legs…diplegia

 Intraventricular hemorrhage, frequently associated with PVL
 Grade I hemorrhage implies only subependymal bleeding ( <10% of ventricular area filled with blood).
 Grade II hemorrhage involves 10-50% of the ventricular area.
 Grade III hemorrhage involves over 50% of the ventricular area.
 Diplegia does not correlate with birth asphyxia



 Tetraplegia: the most catastrophically disabled children, the vast majority being mentally handicapped with epilepsy
 Pathologies include malformations, intrauterine infections, inborn errors of metabolism, and hypoxia ischemia
 Dyskinetic CP: mostly in term infants, usually with evidence of basal ganglia involvement
 Kernicterus: encephalopathy from hyperbilirubinemia
 Full term infants with marked hyperbilirubinemia, or premature infants without marked hyperbilirubinemia
 Hearing loss and movement disorders-dystonia, choreoathetosis
 Kernicterus rare now with improvement in management of hyperbili. and giving anti-D antibodies to Rh negative mothers



Comorbidities
 Several other neurological disabilities can accompany CP, e.g. seizures, mental retardation, behavioral problems, learning disorders, hyperactivity disorders, …but these problems are not implied by the diagnosis of CP

Risk Factors


 Does an abnormal delivery produce an abnormal infant, or does an abnormal infant cause an abnormal delivery???
 Is all CP the result of fetal hypoxia during labor and delivery?....medicolegal issue for obstetricians
 Fetal heart rate monitoring and early C section became standard of care.
 Great improvements in obstetrical and neonatal care over last 30 years…incidence of CP remains unchanged

 CP is primarily a developmental event, with perinatal asphyxia responsible for less than 10% of the cases
 Hypoxic ischemic encephalopathy is only one type of CP
 Neurological Collaborative Perinatal Project (NCPP)
 Prospective study of 5500 pregnant women
 Documenting prenatal and perinatal events
 Following children till 7 years of age
 75% of children with CP had AS of 7-10, those with AS of < 3, 96.1% normal neurologically
 The majority of normal children had similar risk factors to children with CP
 Leading predictors of CP are congenital malformations and birth weight below 2000gm


 Before pregnancy:
 Unusually long or short interval between pregnancies
 mother has long intervals between menses
 During pregnancy
 Congenital malformations
 Birth weight below 2000gm
 Twin gestation
 Mothers with hyperthyroidism or prescribed thyroid hormones or estrogen in pregnancy
 During the perinatal period
 Chorionitis
 Non-vertex or face presentation…cause of CP or marker of preexisting difficulties
 Birth asphyxia in 10% or fewer of the cases


Hypoxic Ischemic Encephalopathy
 American College of Obstetricians and Gynecologists (ACOG) and American Academy of Pediatrics (AAP) Report Jan. 2003:
• Evidence of a metabolic acidosis in fetal umbilical cord
• Early onset of severe or moderate neonatal encephalopathy in infants born 34 wks or more
• Cerebral palsy of the spastic quadriplegia
• Exclusion of other identifiable etiologies
All four must apply
Risk Factors
 Infants born prematurely:
 CP 25-31 times more likely among infants who weigh less than 1500 gms
 The lower the birth weight and gestational age the higher the incidence of CP

Differential Diagnosis
 Consider an alternative diagnosis and evaluate for an underlying cause if:
 Absence of a definite preceding perinatal insult
 Presence of a positive family history of CP
 Developmental regression
 Presence of occulomotor abnormalities, involuntary movements, ataxia, muscle atrophy, or sensory loss
 With muscle weakness: Duchenne/Becker MD, metabolic disorders, especially mitochondrial encephalomyopathies
 With significant dystonia/involuntary movements: dopa responsive dystonia, glutaric aciduria typ 1, pyruvate dehydrogenase deficiency, Lesch-Nyhan syndrome, Rett syndrome
 With significant ataxia: ataxia-telangectasia, GM1 gangliosidosis, X-linked spinocerebellar ataxia, Niemann-Pick disease type C

Investigations
 HISTORY:
 Prenatal history and details of pregnancy
 History of previous abortions, parental consanguinity, and family history
 Perinatal history and details of delivery, APGAR scores
 Developmental history, look for any evidence of regression
 GOOD NEUROLOGICAL EXAM ROUTINELY AS PART OF NEONATAL/CHILD CARE
 Microcephaly
 Poor feeding and communication
 Persistent primitive reflexes, motor stereotypy
 Absence of postural reflexes
 Abnormal obligatory postures
 Visual problems: squints, poor vision…in 11%
 Neonatal head U/S or CT
 MRI always advocated, ideally at 2-3 years of age
 Chromosomal analysis with malformations, dysmorphism or a family history
 Lab investigations as indicated

Management
 Spasticity
 Physical therapy is the mainstay of treatment
 Equipment needs: orthotics, walkers, wheelchairs, …
 Medical treatments
 systematically (benzos, baclofen, dantrolene)
 local treatment such as botulinum toxin injections
 Orthopedics
 Surveillance of hip displacement
 Early detection and treatment of scoliosis
 Recognition and treatment of drooling
 Treatment of seizures
 Specific learning and communication difficulties
 Gastroenterology
 Failure to thrive, less commonly obesity
 Constipation
 Swallowing difficulties and aspiration
 Gastro-esophageal reflux
 Dental caries
 Pulmonology: aspiration, chronic lung disease

Prevention
 Only 9% of patients with CP in the NCPP lacked a major congenital malformation or other intrinsic defect
 Further improvement in neonatal care
 Design of drugs that interfere with excitatory amino acids, such as NMDA antagonists