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Journal of Pediatric Neurology 2004; 02(04): 213-218
DOI: 10.1055/s-0035-1557222
Original Article
Georg Thieme Verlag KG Stuttgart – New York

Peripheral neuropathy in merosin-negative congenital muscular dystrophy

H. Jacobus Gilhuis
1   Department of Neurology and Clinical Neurophysiology, Neuromuscular Centre Nijmegen, University Medical Centre St Radboud, HB Nijmegen, The Netherlands
,
Oebele F. Brouwer
2   Department of Neurology, University Hospital Groningen, RB Groningen, The Netherlands
,
Aad Verrips
3   Department of Neurology, Canisius-Wilhelmina Hospital, GS Nijmegen The Netherlands
,
Machiel J. Zwarts
1   Department of Neurology and Clinical Neurophysiology, Neuromuscular Centre Nijmegen, University Medical Centre St Radboud, HB Nijmegen, The Netherlands
› Author Affiliations
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Further Information

Publication History

22 March 2004

03 June 2004

Publication Date:
29 July 2015 (online)

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Abstract

Peripheral neuropathy in patients with merosin-negative congenital muscular dystrophy (MN-CMD) has been sporadically investigated and has been considered to be motor and demyelinating in nature on the basis of nerve conduction studies. We performed neurophysiologic studies in 12 children with MN-CMD to establish the spectrum and evolution of peripheral nervous system involvement. In our patients, nerve conduction studies for both motor and sensory nerves were near normal in the children younger than six months and abnormal in the older children. The older children had the relatively slowest nerve conduction velocities suggesting a progressive, age-related dysmyelinating neuropathy. We hypothesize that the findings are due to a myelination arrest as a result of insufficient synthesis and maintenance of the peripheral myelin sheath. (J Pediatr Neurol 2004; 2(4): 213–218).

Keywords

merosin-negative congenital muscular dystrophy - peripheral nervous system