Neuropediatrics 1995; 26(1): 3-7
DOI: 10.1055/s-2007-979711
Original articles

© Hippokrates Verlag GmbH Stuttgart

Somatosensory and Visual Evoked Potentials in Congenital Muscular Dystrophy: Correlation with MRI Changes and Muscle Merosin Status

E. Mercuri1 , F. Muntoni1 , Angela Berardinelli1 , Jackie Pennock2 , Caroline Sewry1 , J. Philpot1 , V. Dubowitz1
  • 1Department of Paediatrics and Neonatal Medicine
  • 2The Robert Steiner MRI Unit Royal Postgraduate Medical School, Hammersmith Hospital, Du Cane Road, London W12 ONN, UK
Further Information

Publication History

Publication Date:
19 April 2007 (online)

Abstract

Congenital muscular dystrophy comprises a heterogeneous group of disorders, that have in common an early onset and a dystrophic picture on the muscle biopsy The "pure" form of congenital muscular dystrophy is not associated with severe mental retardation or structural changes in the brain, though white matter changes on brain imaging have been detected in a significant proportion of cases. In this study we evaluated the incidence of sensory abnormalities (somatosensory and visual evoked responses) in a group of 17 patients with "pure" congenital muscular dystrophy and correlated the results of the evoked responses with the presence or absence of white matter changes on brain magnetic resonance imaging. Our results show close correlation between the presence of MRI white matter changes and abnormalities in the sensory evoked potentials. Conversely, all patients with normal brain MRI had normal somatosensory evoked potentials (SEP). Visual evoked potentials were less sensitive than somatosensory evoked potentials in detecting abnormalities in children with white matter changes on MRI. With the recent discovery of deficiency in merosin expression in the skeletal muscle of a subgroup of patients with CMD, we also correlated the presence or absence of white matter changes and the SEP responses with the merosin status.

The results indicate that all merosin-negative patients had abnormal SEP as well as abnormal MRI, whilst no patient with normal merosin expression had an abnormal scan or abnormal SEP The study of evoked potentials in CMD could therefore be of value not only for identifying which patients are more likely to have white matter involvement, but also for subdividing different clinical groups within the CMD category.

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