Prevalence study of cerebral palsy in Hong Kong children.

OBJECTIVES: To investigate the prevalence of cerebral palsy in local children aged 6 to 12 years and to evaluate service utilisation by those children who attend mainstream schools. DESIGN: Cross-sectional survey. SETTING: Mainstream primary schools and special needs schools in Hong Kong. PARTICIPANTS: Headmasters or headmistresses of special needs schools, and various organisations that provide services to children with cerebral palsy in the school year September 2003 to June 2004. MAIN OUTCOME MEASURES: Prevalence of cerebral palsy and support services used by children with cerebral palsy who attend a mainstream school. RESULTS: Of 435 572 children, 578 with cerebral palsy were identified. The overall point prevalence was 1.3 per 1000 children. The age-specific prevalence rate varied from 1.04 to 1.50 per 1000 children. Approximately 38% of children with cerebral palsy attended a mainstream school. Among those studying in special needs schools, 96% attended a school for the physically handicapped or a school for the severely mentally handicapped. Among 219 children with cerebral palsy in mainstream schools, 57 (26%) received educational support, and 134 (61%) received out-patient therapy support. Only 12% received both supporting services. No educational or therapeutic support was received by 26% of children. CONCLUSIONS: Compared with overseas data, the low prevalence of cerebral palsy detected in local children in this investigation may be due to the differences in study design or a genuinely low prevalence. Setting up a cerebral palsy registry could help monitor the local prevalence of this childhood disability more accurately, thereby providing more reliable information for planning support services for this subgroup of children.

Dentatorubral-pallidoluysian atrophy in two Chinese families in Hong Kong.

We report on two Hong Kong Chinese families with dentatorubral-pallidoluysian atrophy. Two children in one family presented with progressive myoclonic epilepsy syndrome, and two children in the other family presented with ataxochoreo-athetoid symptoms. Early-onset childhood dentatorubral-pallidoluysian atrophy involved mental retardation, whereas myoclonic epilepsy was the predominant complaint in later-onset childhood version of the disease. Aspiration pneumonia was common in the late stage of disease. Dentatorubral-pallidoluysian atrophy is an autosomal dominant condition attributed to CAG trinucleotide repeats in the dentatorubral-pallidoluysian atrophy gene. The four children in this series had 63 to 79 CAG repeats. The expanded allele was inherited from the father in both families. One father had 54 CAG repeats and was asymptomatic; the other had 66 repeats and had an unsteady gait. Because the radiological, electroencephalographic, and electrophysiological findings were non-specific, we suggest that DRPLA gene testing should be performed in any child presenting with a variable combination of myoclonic epilepsy, mental retardation or developmental regression, and ataxochoreo-athetosis.