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Autonomic function in Friedreich's ataxia.

T J Ingall1, J G McLeod

  • 1Department of Neurology, Royal North Shore Hospital, St Leonards, NSW, Australia.

Journal of Neurology, Neurosurgery, and Psychiatry
|February 1, 1991
PubMed
Summary
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Autonomic function tests in Friedreich's ataxia patients revealed increased heart rate but normal sympathetic and parasympathetic responses. This aligns with nerve fiber degeneration patterns observed in the disease.

Area of Science:

  • Neurology
  • Autonomic Nervous System Studies
  • Clinical Medicine

Background:

  • Friedreich's ataxia is a rare inherited disease.
  • It primarily affects the nervous system, leading to progressive disability.
  • Autonomic dysfunction can occur in neurological disorders.

Purpose of the Study:

  • To investigate autonomic nervous system function in patients with Friedreich's ataxia.
  • To compare autonomic function test results with those of healthy individuals.
  • To correlate autonomic findings with known pathological changes in Friedreich's ataxia.

Main Methods:

  • Autonomic function studies were conducted on 15 Friedreich's ataxia patients.
  • Results were compared to a control group of 76 healthy subjects.

Related Experiment Videos

  • Specific tests assessed sympathetic and parasympathetic nervous system functions.

    • Main Results:

    • Patients with Friedreich's ataxia showed an elevated resting supine heart rate.
    • This increase was attributed to cardiac abnormalities.
    • Tests for sympathetic and parasympathetic functions were within normal limits.

    Conclusions:

    • Autonomic function appears largely preserved in Friedreich's ataxia, apart from cardiac effects.
    • Normal autonomic test results are consistent with pathological findings of selective nerve fiber degeneration.
    • The study suggests sparing of small myelinated and unmyelinated fibers in Friedreich's ataxia.