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Related Experiment Videos

Evoked potentials in hereditary spastic paraplegia

I M Sawhney1, S K Bansal, P K Upadhyay

  • 1Department of Neurology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

Italian Journal of Neurological Sciences
|September 1, 1993
PubMed
Summary

Abnormalities in visual evoked response (VER) and brainstem auditory evoked response (BAER) were found in patients with hereditary spastic paraplegia (HSP). These findings suggest demyelination and could aid in diagnosing HSP.

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Area of Science:

  • Neuroscience
  • Clinical Neurology
  • Neurophysiology

Background:

  • Hereditary spastic paraplegia (HSP) is a group of inherited neurological disorders.
  • Clinical presentation of HSP typically involves progressive lower limb spasticity and weakness.
  • Subtle or subclinical central nervous system involvement may occur in HSP.

Purpose of the Study:

  • To investigate the utility of neurophysiological tests in diagnosing hereditary spastic paraplegia (HSP).
  • To assess for subclinical central nervous system involvement using visual evoked response (VER) and brainstem auditory evoked response (BAER) in HSP patients.
  • To correlate neurophysiological findings with the pathophysiology of HSP.

Main Methods:

  • Recorded pattern reversal visual evoked response (VER) and monaural brainstem auditory evoked responses (BAER) in 25 patients diagnosed with hereditary spastic paraplegia (HSP).

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  • Patients' ages ranged from 15 to 52 years, with symptom duration from 6 months to 9 years.
  • Evaluated both sides for evoked potential abnormalities.

    • Main Results:

    • Prolonged P100 latency in VER was observed in 6 patients (24%).
    • Abnormalities in BAER were detected in 13 patients (52%).
    • None of the patients exhibited clinical signs of brainstem involvement.

    Conclusions:

    • Abnormalities in VER and BAER suggest demyelination and fiber loss in central conduction pathways in HSP patients.
    • These neurophysiological tests can serve as valuable diagnostic tools for hereditary spastic paraplegia.
    • Evoked potential testing may reveal subclinical neurological deficits in HSP.