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Research Article| Volume 16, ISSUE 1, P65-70, January 1994

Two patients with distal muscular dystrophy and autonomic nerve dysfunction

DOI:https://doi.org/10.1016/0387-7604(94)90116-3
Two patients with distal muscular dystrophy and autonomic nerve dysfunction
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      Abstract

      Two female patients with distal muscular dystrophy (Miyoshi) are reported. Neurological examination revealed marked weakness and a low skin temperature of both lower legs, especially over the gastrocnemius muscle. Biopsy specimens of the anterior tibial muscle exhibited myopathological features compatible with a dystrophic change and showed positive dystrophin reactions with all six antibodies used. Autonomic nerve studies in both patients, including laser Doppler flowmetry (LDF), component analysis of the cardiographic R-R interval and a sympathetic skin response (SSR) test, demonstrated marked abnormalities, such as sensitive vasoconstrictive responses, a suppressed peak of low frequency components and an absence of SSR, respectively, compared with findings in healthy controls. Sympathetic nerve blocking by means of epidural anesthesia produced clinical improvement and a marked decrease in the serum CK level in one patient. Although the etiology of autonomic nerve disturbances in these cases remains to be elucidated, a positive immunoreactivity of nerve growth factor receptor (NGFR) implies the possibility of some unknown sympathetic neurovascular disorder involving muscle degeneration.

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      Article info

      Publication history

      Accepted: August 15, 1993
      Received: May 31, 1993

      Identification

      DOI: https://doi.org/10.1016/0387-7604(94)90116-3

      Copyright

      © 1994 Elsevier Science B.V. All rights reserved. Published by Elsevier Inc.

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