Article

AIM: To report on the epidemiology of the brain white matter disorders of
children identified via a national prospective study.
METHOD: Since 1997 a study
of UK children with progressive intellectual and neurological deterioration
(PIND) has used the British Paediatric Surveillance Unit system to identify
children with progressive neurodegenerative disease.
This paper reports on
children in the study with brain white matter disorders. RESULTS: Between May
1997 and November 2014 the PIND study identified 349 children with diagnosed
leukodystrophies, giving an estimated UK lifetime risk of 31/million live births.
There were 18 specific diseases in the group and relatively large numbers of
affected children came from consanguineous Pakistani families. In addition there
were 454 children with genetic leukoencephalopathies - in this group there were
38 diseases. 5.8% of children with scan evidence of brain white matter disorders
did not receive a specific diagnosis. INTERPRETATION: These unique
prospectively-obtained national data avoid the selection bias inherent in reports
from single centres. White matter disorders of the central nervous system
comprise more than half of UK paediatric neurodegenerative diseases meeting the
PIND criteria. This paper reports the lifetime risk/million live births for the
commonest leukodystrophies, providing a basis for comparison with future studies.
CI - (c) 2016 Mac Keith Press.

Langue : ANGLAIS