PDF icon Download PDF
Neuropediatrics 1995; 26(3): 156-162
DOI: 10.1055/s-2007-979746
Original articles

© Hippokrates Verlag GmbH Stuttgart

Minor Neurological and Perceptuo-Motor Deficits in Children with Congenital Muscular Dystrophy: Correlation with Brain MRI Changes

E. Mercuri1 , L. Dubowitz1 , A. Berardinelli1 , J. Pennock2 , M. Jongmans3 , S. Henderson3 , F. Muntoni1 , C. Sewry1 , J. Philpot1 , V. Dubowitz1
  • 1Department of Paediatrics and Neonatal Medicine London, UK
  • 2The Robert Steiner MRI Unit, Royal Postgraduate Medical School, Hammersmith Hospital, London, UK
  • 3Institute of Education, University of London, UK
Further Information

Publication History

Publication Date:
19 April 2007 (online)

Also available ateRef
 PDF Download  Permissions and Reprints
Preview

Abstract

Diffuse white matter changes on magnetic resonance imaging (MRI) have been a consistent feature in some children with the "pure" form of congenital muscular dystrophy (CMD) in which there are no structural changes in the brain or severe mental retardation. The aim of this study was to assess fine motor and perceptuo-motor abilities in children with 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 visual-motor integration, Zurich Neuromotor test) which have already been used to detect minor neurological signs related to white matter changes. The cohort was then divided in two groups for analysis depending on the presence or the absence of diffuse white matter changes. A significant difference was found for all the tests between the group of the CMD children with normal MRI and the group with diffuse white matter changes. The manual dexterity and the Zurich Neuromotor tests showed a greater sensitivity than the test of visual-motor integration, which had some false negatives. It is of interest that in the group with diffuse 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 extent 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 neurological soft signs are a consistent feature in CMD children with diffuse MRI changes but not with normal MRI. We suggest that a more detailed neurological examination for the detection of these abnormalities may lead to more specific supportive help for everyday life and school performances in these children. The concordance of our results with MRI findings would also suggest that these tests might provide an additional help in identifying the different subgroups of CMD.

Key words

Congenital muscular dystrophy - Brain - Merosin - MRI