CEREBRAL PALSY MANAGEMENT OF CHILDREN WITH CEREBRAL PALSY The term cerebral palsy refers to motor impairments that result from an insult to the immature motor cortex and/or its motor pathways. I would like to dedicate this article to Dr Leila J Arens, whose passion for manag- ing children with cerbral palsy spans more than 4 decades, and has inspired so many of us to work in this field. The neurological lesion causing cerebral palsy (CP) is static, but the clinical con- dition changes over time. CP has a wide clinical spectrum with a number of aeti- ologies. It is not sufficient just to make the diagnosis of CP; the term needs further qualification by a more detailed description of the clinical condition. There are different types of CP, ranging in severity and associated with a variety of prob- lems. Doctors at primary and secondary level often feel inadequate, due to the complex and diverse nature of the CPs and their associated problems. There is also no magic cure, but rather intervention to allow the patient to reach maxi- BARBARA LAUGHTON mum function and potential and to prevent further complications. This article MB ChB, DCH (SA), FCPaed (SA) aims to give a logical and sound approach to the assessment and management Registered Developmental of the common problems and complications in patients with CP. These children Paediatrician may then be referred when a need for specialised intervention is identified, if Paediatric Neurology Clinic they are deteriorating on current management, or if there is doubt about the diagnosis or management plan. Tygerberg Children’s Hospital Cape Town ASSESSMENT OF CHILDREN WITH CEREBRAL PALSY Dr Laughton works as a consultant at Careful assessment of a patient with CP Tygerberg Children’s Hospital. Her special (Table I) will elicit problems specific to Table I. Assessment of a child interest is in cerebral palsy and screening the individual child and allow the prac- with CP the high-risk premature infant. titioner to plan the appropriate manage- • Confirmation of the diagnosis of ment. The following questions should be CP asked: • Excluding the mimics Is it cerebral palsy? • Defining the aetiology • The type of cerebral palsy There must be evidence of an upper • The severity motor neuron (UMN) lesion, with brisk • Associated medical problems reflexes and possibly clonus; there • Level of functioning should also be increased tone or persist- • Short- and long-term goals ent primitive reflexes. Hypotonic CP has many mimics and this diagnosis is best made at specialist centres. What could mimic CP? A number of CNS disorders mimic CP.1 These include metabolic and genetic con- ditions. Be very careful of making the diagnosis of CP if: • the history is not clearly compatible with the common causes of CP • the child's development is regressing • new clinical signs appear • the clinical signs fluctuate during the day • there is abnormal posturing • there is a ‘family history’ of CP. 434 CME August 2004 Vol.22 No.8 CEREBRAL PALSY What was the aetiology? Neurodevelopmental treatment-trained Table II. Common associated (NDT) therapists have been trained in There is often a clear history of a CNS problems/complications insult or high-risk situation like prema- the complexities of movement at the ture birth, but frequently no identifi- • Intellectual disability different gross motor milestones and able cause can be found. • Epilepsy or seizures are invaluable in the management of •Visual impairment these patients. Where resources are What type is it? • Hearing impairment limited, teaching the caregiver to prac- CP is divided into different types • Feeding/swallowing problems tise these techniques on a daily basis according to the clinical presentation: • Gastro-oesophageal reflux is the primary goal, with monthly visits spastic, hypotonic, dyskinetic, ataxic • Failure to thrive to the therapist if possible. or mixed. It is further qualified accord- • Constipation ing to the distribution of the limbs and • Dental caries Important aspects of the extent of involvement, e.g. quadri- •Orthopaedic complications physiotherapy plegia, hemiplegia or diplegia, or the • Financial Hemiplegia type of movement. The section on CP • Mental health of patients and An infant using only one hand needs 2 in Coovadia and Wittenberg has a carers urgent referral to a therapist before good description of the different types. the infant is upright and has learned The type of CP is not always pure and What are the short- and to compensate totally with the unaffect- should be reviewed regularly, as the long-term goals for this ed side. Weight bearing on the affect- clinical condition may change over patient? ed arm is possible while the infant is time as the brain matures (e.g. an Decisions should be made by a multi- learning to come up to sitting and infant with spastic quadriplegic CP disciplinary team, which should crawling. The prognosis is good for may develop dystonic posturing later; include the children and their family. the affected arm to weight bear and children with hypotonia may evolve act as an assisting arm, even if the into the ataxic type later, and athetoid MANAGEMENT OF CEREBRAL child is unable to fasten a button. movements usually appear later). PALSY Severe spastic quadriplegia How severe is the Management is aimed at minimising Supine lying should be avoided if pos- condition? the progressive deformity, improving sible as this reinforces the abnormal This is a clinical description based on the child's functional outcome and extended position, with the child star- the physical ability of the child. The managing the associated problems. ing at the ceiling. Windswept position traditional categories are: minimal in supine should also be avoided to (motor signs present but no functional Physical rehabilitation prevent scoliosis and dislocated hip. impairment), mild (limited impairment), moderate (obvious impairment, usually Physiotherapy Encourage the caregiver to place the requires assistive devices for ambula- Physiotherapy is the mainstay of treat- child on its side, lying with a pillow tion), severe (little purposeful voluntary ment for the majority of children with between the legs. This will also bring action).3 CP. The role of physiotherapy in the hands to the midline to play with a improving the outcome (e.g. improving toy. What are the associated a spastic quadriplegic’s mobility to problems? enable them to walk) is controversial. Prone lying over a longitudinal pillow Table II lists the common problem associ- However, there is clearly no doubt with weight bearing on the forearms ated with CP that should be addressed about the role of physiotherapy in should alternate with side lying. during history taking and examination. maintaining the current function of the In some cases the associated problem child and reducing the incidence and Never lose the feet. Always teach a may be more problematic than the CP. severity of further complications like caregiver to maintain 90 degrees of joint contractures, deformities and dis- What is the child's level of locations. The success of medical and ankle dorsiflexion so that even a functioning? surgical interventions for CP is also severely affected child can be placed in the standing position, if only for The description should include other highly dependent on rehabilitative deficits, and the level of functioning with exercises to optimise results.4 transfer from bed to chair. Lifting a and without assistive devices, e.g. heavy child is difficult. patient is educable, able to walk using Physiotherapy is not just ‘stretching ankle-foot orthoses and holding onto the exercises’. It is orientated towards Maintaining passive joint range is wall, otherwise crawls; moderate hear- activities of daily living by improving important for washing, especially hip ing loss corrected with hearing aids; important components of movement, abduction in side lying for girls during speech is understandable, but slow and e.g. improving trunk control to menstruation. deliberate. improve a child’s walking ability. August 2004 Vol.22 No.8 CME 435 CEREBRAL PALSY Speech therapy and may increase oral secretions and Correct supportive seating can main- General movement difficulties, as well care ought to be taken in patients with tain good posture and positioning and as involvement of the oral muscles, may swallowing problems. assist with improved function. Buggies contribute to the failure to develop com- are most commonly used for young Baclofen (Lioresal) (10 mg municative intent, and cause speech children. tablet) delay and difficult feeding. Intervention should start early. The basic principles Sedation is less than with diazepam Surgical intervention are to introduce a therapeutic feeding and this drug is useful in higher Orthopaedic surgery programme that includes positioning of functioning patients. It has variable the child, and techniques to facilitate results but is worth a trial. Start at Specialised paediatric orthopaedic mouth closure and appropriate chewing 2.5 - 5 mg per day and increase every surgeons are an essential part of the movements, plus blowing, sucking, lick- 4 - 7 days to maximum doses of 30 mg CP management team. Their knowl- ing etc. Receptive language develop- (children 2 - 7 years of age) to 60 mg edge of the dynamics of multilevel ment is often much better than expres- (children 8 years or older) per day in 3 joint involvement in functional mobility sive language development and these divided doses. It is very poorly is invaluable in determining the most children’s cognitive function should not absorbed orally and high doses are appropriate management. Once there be assessed on expressive language usually needed. It may cause sedation, are contractures or joint abnormalities, alone. Communication may be assisted ataxia, hypotension and paraesthesia. surgical intervention is the only treat- by augmentative alternative communica- Some patients may have increased ment available. Indications for sur- tion like communication boards or seizures. Abrupt discontinuation can gery include improving the quality of 5 home-made pictures to point at.