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Peripheral neuropathy in myotonic dystrophy: A nerve biopsy study
Author(s) -
Cros Didier,
Harnden Patricia,
Pouget Jean,
Pellissier Jean Françlois,
Gastaut Jean Louis,
Serratrice Georges
Publication year - 1988
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410230508
Subject(s) - remyelination , myotonic dystrophy , sural nerve , medicine , atrophy , myelin , pathology , axonal degeneration , anatomy , nerve biopsy , nerve fiber , peripheral neuropathy , central nervous system , endocrinology , diabetes mellitus
Sural nerve biopsies from 13 unselected myotonic dystrophy patients and 6 normal controls were studied morphometrically. The myelinated fiber density was reduced in 11 of the 13 myotonic dystrophy patients, with preferential loss of large myelinated fibers. Unmyelinated fiber densities and diameters were normal. Teased fiber studies commonly revealed focal areas of remyelination and abnormal wrinkling of the myelin sheath. Measurement of internodal length disclosed features of both axonal regeneration and focal demyelination‐remyelination. These findings are consistent with a chronic axonopathy of moderate severity, possibly due to axonal atrophy.