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Detection of early abnormalities in the mucopolysaccharidoses by the use of visual and brainstem auditory evoked

A Perretti1, A Petrillo, L Pelosi

  • 1Department of Clinical Neurophysiology, 2nd School of Medicine, University of Naples, Italy.

Neuropediatrics
|May 1, 1990
PubMed

Insights

This study found that flash visual evoked potentials (FVEPs) and brainstem auditory evoked potentials (BAEPs) can detect early neurological abnormalities in children with mucopolysaccharidoses (MPSs), even if they appear neurologically normal.

Area of Science:

  • Neuroscience
  • Pediatrics
  • Genetics

Background:

  • Mucopolysaccharidoses (MPSs) are a group of rare genetic disorders.
  • Neurological complications can occur in MPSs, but early detection is challenging.
  • Electrophysiological assessments are valuable for evaluating nervous system function.

Purpose of the Study:

  • To investigate the utility of flash visual evoked potentials (FVEPs) and brainstem auditory evoked potentials (BAEPs) in children with MPSs.
  • To identify early electrophysiological abnormalities in neurologically normal patients with MPSs.

Main Methods:

  • Performed FVEPs and BAEPs on 13 children diagnosed with MPSs (aged 3-17 years).
  • Analyzed the electrophysiological data for abnormalities.

Main Results:

  • Abnormalities were detected in FVEPs for 6 out of 13 patients.
  • Abnormalities were detected in BAEPs for 9 out of 13 patients.
  • Most patients with abnormalities showed no apparent neurological deficits.

Conclusions:

  • FVEPs and BAEPs are sensitive tools for detecting subclinical neurological dysfunction in children with MPSs.
  • Electrophysiological testing can aid in the early identification of potential complications in MPS patients.
  • These findings support the routine use of evoked potentials in the management of MPS.

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