Affiliations: Department of Neurology and Clinical Neurophysiology,
Neuromuscular Centre Nijmegen, University Medical Centre St Radboud, HB
Nijmegen, The Netherlands | Department of Neurology, University Hospital
Groningen, RB Groningen, The Netherlands | Department of Neurology, Canisius-Wilhelmina Hospital,
GS Nijmegen The Netherlands
Note: [] Correspondence: H. Jacobus Gilhuis, M.D., Neuromuscular Centre
Nijmegen, Department of Neurology and Clinical Neurophysiology, University
Medical Centre St Radboud, PO Box 9101, 6500 HB Nijmegen, The Netherlands. Tel:
+ 31-24-3613394, fax: + 31-24-3617018. E-mail: [email protected]
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
