Cerebral Palsy. What is Cerebral Palsy? Clues to Diagnosis of Cerebral Palsy 12/30/2012

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1 What is Cerebral Palsy? Cerebral Palsy Hamza Alsayouf,MD American Board Of neurology with Special Qualification in Child Neurology American Board of Pediatric Neurology In 2005, a committee of the American Academy for Cerebral Palsy and Developmental Medicine (AACPDM), led by Peter Rosenbaum, defined CP as: a group of disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. The motor disorders of cerebral palsy are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior, and/or by a seizure disorder. In the 6 th edition of the International Classification of Diseases handbook, there are over 50 different classifications of CP. The incidence of CP is considered to be 2 to 2.5/1000 live births. Diagnosis usually involves waiting for the definite and permanent appearance of specific motor problems Clues to Diagnosis of Cerebral Palsy Delayed milestones; persistence of primitive reflexes; presence of pathologic reflexes; failure to develop protective reflexes such as parachute response, asymmetric movements. No progressive neurological disease and NO loss of milestones should be present. Beware of changes in exam that may result from growth and maturity! 1

2 Cerebral Palsy It is not unusual for child with cerebral palsy to have hypotonia initially and then hypertonia (why). However, if a child has hypertonia initially, then becomes hypotonic, a neurodegenerative disease should be searched (why). Spastic : 70 % Classification of Cerebral Palsy Syndromes Bilateral spasticity Spastic diplegia : Prematurity, commonest. Spastic quadriplegia: most severe form,hie. Unilateral spasticity Spastic hemiplegia: stroke,seizure most common in this form. Monoplegia and Triplegia: uncommon. Extrapyramidal (dyskinetic) % Bradykinesia Dystonia Choreoathetosis: Kernicterus. Hemiballismus Hypotonia Ataxic Mixed The Gross Motor Function Classification System(GMFCS) Provides information about the severity of the movement problems based on the child s motor abilities and their need for walking frames, wheelchairs and other mobility devices. The GMFCS define five levels of function for four age groups : before 2 years, 2-4 years, 4-6 years, 6-12 years, with recent addition of years. There is a strong correlation between this classification and the World Health Organization International Classification of Impairments, Disabilities and Handicaps, and is also easy to use. There are five levels within the GMFCS: children in Level I and II walk independently; children in Level III need walking frames or elbow crutches and children in Level IV and V require a wheelchair 2

3 Causes of Cerebral Palsy Prenatal Abnormalities commonest % Congenital Abnormalities 1 st trimester Teratogens Genetic syndromes Chromosomal abnormalities Brain malformation 2 nd 3 rd trimester Intrauterine infections Problems in fetal/placental function Causes of Cerebral Palsy Perinatal : % sepsis/cns infection Labor and delivery Asphyxia Prematurity Postnatal: 10 % meningitis traumatic brain injury Toxins,Biochemical defects Not obvious 30 % Differential diagnosis of Cerebral Palsy Inborn errors of metabolism Neurodegenerative diseases Tay Sachs disease Leukodystrophies Retts Syndrome Hereditary abnormalities Trisomies 13 and 18 Hereditary spastic paraplegia Familial dystonia Dopa responsive dystonia Important practice points Search for the cause of CP as this is likely to be helpful for parents and may assist with genetic counselling. From the age of about 2 years it is possible to predict likely motor outcome. Advice can be given about the likelihood of independent walking or whether the child will be wheelchair-dependent. Ensuring an up-to-date immunization schedule is essential. Yearly influenza vaccine is important for those with severe motor impairment. Regular hip surveillance is imperative to prevent the secondary problems of subluxation and dislocation. Consider the needs of the family, for example, has suitable lifting equipment been provided for a nonambulant child; has respite been discussed; do the needs of siblings require attention? 3

4 When a child with CP presents with pain or irritability, consider the following possibilities: Management of the consequences of the motor disorder intercurrent infection (particularly urinary tract infection) dental disease pathological fracture gastro-oesophageal reflux and/or gastritis/oesophagitis constipation renal stones (increased risk if on topiramate) subluxating and dislocated hips sleep deprivation, which in turn may be caused by pain or spasm increased intracranial pressure (many children have ventriculoperitoneal shunts) nonaccidental injury (under-reported in this group) changes in home environment, school or family that may be impacting on wellbeing Drooling (dribbling or poor saliva control) can be improved with speech pathology intervention, or by the use of anticholinergic medication or surgery Incontinence: bowel and bladder control may be achieved late due to cognitive deficits or difficulty in accessing the toilet because of physical disability and the inability to communicate. Detrusor overactivity may cause urgency and frequency. Advice about continence aids is useful Undescended testes usually require scrotal orchidopexy. The testes may be in the normal position at birth but ascend with time due to chronic spasm of cremastermuscle. Orthopaedic problems: surgery is mainly undertaken on the lower limbs, but is occasionally helpful for the upper limbs. Physiotherapists are essential in the postoperative phase. Gait laboratories are useful in planning the surgical program for ambulant children The hip: nonwalkers and those partially ambulant are at risk for hip dislocation, which causes pain and difficulty with perineal hygiene. Early detection is vital and hip X-rays should be performed at regular intervals according to hip surveillance guidelines.11 If there is evidence of subluxation or dislocation, children should be referred for an orthopaedic opinion The knee: flexion contractures at the knee may require hamstring surgery to improve gait pattern, or the ability to stand for transfers. The ankle: toe walking is treated conservatively in young children, with orthoses, inhibitory casts and botulinum toxin. Older children with equinus deformity require a definitive correction Describing Cerebral palsy Multilevel surgery: some children require surgery at several different levels, for example, hip, knee and ankle. This is called single event multilevel surgery and is of most benefit to children who walk independently or with the assistance of crutches. Scoliosis correction is sometimes necessary. Spasticity management aims to improve function, comfort and care. Options include: Oral medications (eg. diazepam and baclofen). Inhibitory casts to increase joint range, mainly used below the knee for equinus deformity. Botulinum toxin A to reduce localised spasticity. Intrathecal baclofen administered by an implanted pump suitable for a small group of children with severe generalised spasticity and/or dystonia Selective dorsal rhizotomy, an uncommon procedure whereby specific posterior spinal roots are sectioned to reduce spasticity. 4

5 Spastic Quadriplegia Hemiplegia Athetoid Cerebral Ataxia Cerebral Palsy 5

6 Diplegia Other abnormalities often associated with CP Impairments in: Vision Hearing Sensory Language Cognition No cure!!! Treatments for Cerebral Palsy Physical therapy, Occupational therapy Braces, mobility tools Benzodiazepines for decreasing spascity Baclofen pump for decreasing spascity Treating constipation, healthy diet Botox injections Lengthening of tendons Dorsal rhizotomies Epilepsy occurs in almost half of children with CP. Careful management is required General Prognosis for Cerebral Palsy Mixed spastic and extrapyramidal cerebral palsy: almost never resolved. Spastic hemiplegic cerebral palsy: almost walk by 2 years. Spastic diplegia: more than 50% walk by age of three years. Spastic quadriplegia: 33% walk by age of three years. 6

7 Conclusion Cerebral palsy is a diagnosis of pattern recognition Cerebral palsy with its associated conditions and pseudo degeneration, may be initially difficult to differentiate from a true slowly progressive neurodegenerative disease Accurate diagnosis is important for genetic counseling Athetoid Cerebral Palsy Spastic Cerebral Palsy Gait QUESTIONS? 7

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