Peripheral neuropathy in merosin-negative congenital muscular dystrophy
| Autor: | Aad Verrips, Oebele F. Brouwer, Machiel J. Zwarts, H. Jacobus Gilhuis |
|---|---|
| Rok vydání: | 2015 |
| Předmět: |
Merosin-negative congenital muscular dystrophy
Pathology medicine.medical_specialty business.industry Peripheral myelin Sensory system medicine.disease medicine.anatomical_structure Peripheral neuropathy Peripheral nervous system Pediatrics Perinatology and Child Health Congenital muscular dystrophy Medicine In patient Neurology (clinical) business Nerve conduction Neuroscience |
| Zdroj: | Journal of Pediatric Neurology. :213-218 |
| ISSN: | 1875-9041 1304-2580 |
| DOI: | 10.1055/s-0035-1557222 |
| Popis: | 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). |
| Databáze: | OpenAIRE |
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