MINOR NEUROLOGICAL AND PERCEPTUOMOTOR DEFICITS IN CHILDREN WITH CONGENITAL MUSCULAR-DYSTROPHY - CORRELATION WITH BRAIN MRI CHANGES

Diffuse white matter changes on magnetic resonance imaging (MRI) have been a consistent feature in some children with the ''pure'' form of c ongenital muscular dystrophy (CMD) in which there are no structural ch anges in the brain or severe mental retardation. The aim of this study was to assess fine motor and perceptuo-motor abilities in children wi th CMD with and without MRI changes. Twenty-two children with ''pure'' CMD were investigated with a standard neurological examination and a battery of tests (Manual dexterity from the Movement ABC, test of visu al-motor integration, Zurich Neuromotor test) which have already been used to detect minor neurological signs related to white matter change s. The cohort was then divided in two groups for analysis depending on the presence or the absence of diffuse white matter changes. A signif icant difference was found for all the tests between the group of the CMD children with normal MRT and the group with diffuse white matter c hanges. The manual dexterity and the Zurich Neuromotor tests showed a greater sensitivity than the test of visual-motor integration, which h ad some false negatives. It is of interest that in the group with diff use white matter changes the presence of contractures or weakness did not seem to affect the quality of the performance; all these children scored abnormally on the test, irrespective of the severity or the ext ent of contractures and weakness. In contrast, in children with normal MRI severe contractures and weakness did affect the performances. Our results demonstrate that perceptuo-motor difficulties and minor neuro logical soft signs are a consistent feature in CMD children with diffu se MRI changes but not with normal MRI. We suggest that a more detaile d neurological examination for the detection of these abnormalities ma y lead to more specific supportive help for everyday Life and school p erformances in these children. The concordance of our results with MRI findings would also suggest that these tests might provide an additio nal help in identifying the different subgroups of CMD.