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Related Experiment Videos

Central conduction and autonomic nervous function in HMSN I.

G Solders1, T Andersson, A Persson

  • 1Department of Clinical Neurophysiology, Huddinge University Hospital, Sweden.

Muscle & Nerve
|November 1, 1991
PubMed
Summary
This summary is machine-generated.

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Central nervous system (CNS) conduction and autonomic nervous function were assessed in patients with hereditary motor and sensory neuropathy type I (HMSN I). Findings suggest that impaired central conduction and autonomic dysfunction are common in HMSN I.

Area of Science:

  • Neurology
  • Neurophysiology

Background:

  • Hereditary motor and sensory neuropathy type I (HMSN I) is a demyelinating peripheral neuropathy.
  • Central nervous system (CNS) involvement and autonomic dysfunction are not well-characterized in HMSN I.

Purpose of the Study:

  • To investigate central nervous system (CNS) conduction and autonomic nervous function in patients with HMSN I.
  • To determine the prevalence of central conduction abnormalities and autonomic dysfunction in this patient cohort.

Main Methods:

  • Central motor conduction time (CMCT) was measured using magnetic brain stimulation and electrical nerve root stimulation.
  • Somatosensory evoked potentials (SEPs) and visual evoked potentials (VEPs) assessed central sensory and visual pathways.
  • Autonomic function was evaluated via R-R variation testing (parasympathetic) and sympathetic skin response (SSR) testing (sympathetic sudomotor activity).

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Main Results:

  • Five of 15 patients exhibited prolonged CMCT.
  • VEPs were abnormal in 2 patients; SEPs were mostly unassessable or normal/slightly prolonged.
  • Autonomic dysfunction was noted in 8/15 patients (low R-R variation) and 5/15 patients (pathological SSR).

Conclusions:

  • Impaired central conduction, including motor and visual pathways, is observed in a subset of HMSN I patients.
  • Autonomic dysfunction, affecting both parasympathetic and sympathetic systems, is a significant finding in HMSN I.
  • These results indicate that CNS and autonomic involvement are not uncommon in HMSN I, suggesting a more systemic impact of the neuropathy.