White matter abnormalities in congenital muscular dystrophy

Central nervous system (CNS) characteristics were examined in seventeen patients with autosomal recessive classic or “pure” congenital muscular dystrophy (CMD). In three patients, neuroradiological examination (CT/MRI) indicated hypodense white matter areas. Two out of these three patients had epile...

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Bibliographic Details
Published in:Journal of the neurological sciences 1995-04, Vol.129 (2), p.162-169
Main Authors: Leyten, Q.H., Gabreëls, F.J.M., Renier, W.O., van Engelen, B.G.M., ter Laak, H.J., Sengers, R.C.A., Thijssen, H.O.M.
Format: Article
Language:English
Subjects:
(Sub)normal intelligence
Adolescent
Biological and medical sciences
Brain - diagnostic imaging
Brain - pathology
Cerebral white matter hypodensity
Child
Child, Preschool
Congenital muscular dystrophy
Diseases of striated muscles. Neuromuscular diseases
Electroencephalography
Epilepsy
Epilepsy - etiology
Female
Follow-Up Studies
Humans
Infant
Infant, Newborn
Magnetic Resonance Imaging
Male
Medical sciences
Muscular Dystrophies - complications
Muscular Dystrophies - congenital
Muscular Dystrophies - diagnostic imaging
Muscular Dystrophies - pathology
Neurology
Retrospective Studies
Tomography, X-Ray Computed
Type 1 congenital muscular dystrophy
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Description
Summary:Central nervous system (CNS) characteristics were examined in seventeen patients with autosomal recessive classic or “pure” congenital muscular dystrophy (CMD). In three patients, neuroradiological examination (CT/MRI) indicated hypodense white matter areas. Two out of these three patients had epilepsy (seizures and epileptic discharges on their EEG). Only two of the remaining patients had epileptic EEG discharges, but without clinical seizures. By comparing our results to data in the literature, we could conclude that the classic or “pure” form of CMD can be subdivided into two subtypes, i.e. those with and those without white matter hypodensities. A mild form of epilepsy or an epileptic predisposition on EEG can be part of the subtype with white matter hypodensities.
ISSN:0022-510X
1878-5883
DOI:10.1016/0022-510X(94)00264-O