Publication: Ultrastructural relationship of quadriceps muscle degeneration with a distant peroneal nerve conduction in human myotonia dystrophica
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Date
1987
Authors
Sinha, Akhouri A. ; Olson, Norman D. ; Nuttall, Frank Q.
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Publisher
Murcia : F. Hernández
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DOI
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info:eu-repo/semantics/article
Description
Abstract
The association of motor nerve conduction
velocity (MNCV) to (1) duration of symptoms, (2) deep
tendon reflex responses, (3) clinical muscle atrophy, and
(4) ultrastructure of quadriceps muscle was studied in 18
patients with myotonia dystrophica of Steinert and nine
normal controls. These patients had neither diabetes
mellitus nor any other type of muscle dystrophy.
Ultrastructural features of muscle fibers and intercellular
spaces between atrophic fibers provided a basis for
identifying degenerative changes and evaluating them
semi-quantitatively. Our study indicates presence of an
association between the pattern of muscle degeneration
and both MNCV (correlation coefficients, y=+0.60)
and duration of symptoms (y=-0.62), but not between
MNCV and duration of symptoms (y=+0.28). Further
analysis of the association between the degeneration of
quadriceps and the MNCV of a distant peroneal nerve
(which does not innenate quadriceps) suggested that the
systemic nerve degeneration occurred in some groups of
myotonia patients. Our study indicates that while in
some patients the muscle degeneration may have been
associated with the impairment of neurogenic elements,
in others it occured in the absence of any MNCV
abnormality. Our findings favor the role of both
neuropathic and myopathic factors in the muscle
degeneration seen in myotonia dystrophica.
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