Hemiplegic cerebral palsy : clinical presentation and aetiology

Abstract

Cerebral palsy is an umbrella term for motor impairments that share features of a non-progressive brain injury, acquired during the early stages of its development. These motor impairments can have different aetiologic backgrounds, occurring in the prenatal, perinatal or postnatal period. Cerebral palsy is the most common cause of childhood onset, lifelong disability in many countries. The motor impairment is classified as unilateral/ hemiplegic or bilateral. It is often associated with visual- and hearing deficits as well as epilepsy and cognitive impairment. These associated impairments can be more disabling than the motor disturbance itself. Epilepsy is common in cerebral palsy, present in 30-40 % of cases. Epilepsy is related to the underlying brain lesion. Children with cerebral palsy, epilepsy and intellectual disability constitute a large patient group in paediatric neurology.

This thesis contains five clinical studies. The first part of the thesis comprises three clinical studies in which we described the motor impairments, and associated impairments, in a population-based group of children with hemiplegia/unilateral cerebral palsy after cerebral infection. In study I we found a high frequency of epilepsy and intellectual disability among the participants. In study II six children and 15 caregivers in this group were interviewed regarding the child`s ability to participate in age-related activities. All reported great difficulties participating in age-related activities. Hygiene issues hindered children with a non-Swedish cultural background from participating in camps or swimming. In study III the severity of the motor disability in these children was assessed using the Movement Assessment Battery for Children and the Paediatric Disability Inventory. Balance and hand function were impaired in all. Motor difficulties dominated during pre-school years, difficulties with social and communication skills predominated in school-age children.

For the second part of the thesis we studied a population-based group of hemiplegic children, with different aetiologic backgrounds. In study IV we investigated both aetiologic background factors, clinical presentation including brain imaging, electroencephalogram (EEG) and epilepsy found in each child. Risk factors for cerbral palsy were present in 16 of the 21 participants. Risk factors are important for early identification. Any child with these risk factors should be offered a check-up by a paediatrician or paediatric neurologist. Epilepsy was common and associated with combined grey and white matter injuries. In study V we investigated intellectual disability and its association with epilepsy and brain imaging in this population-based group of hemiplegic children. Intellectual disability was present in 38 % and uneven cognitive profiles in 57%. Nine of the children developed epilepsy.

Together, these studies show that epilepsy, intellectual disability and uneven cognitive profiles are common in hemiplegic cerebral palsy. Individual cognitive assessment is recommended before school start.