BAEP changes in Leber's hereditary optic atrophy: further confirmation of multisystem involvement

Acta Neurol Scand. 1990 Apr;81(4):349-53. doi: 10.1111/j.1600-0404.1990.tb01569.x.


A neurophysiological study of 11 patients belonging to 5 families affected by Leber's hereditary optic atrophy is reported. Electromyography, nerve conduction velocities and somatosensory evoked potentials were normal. Visual evoked potentials were absent or delayed, desynchronized and reduced in amplitude. Brainstem auditory evoked potentials were anomalous in 64% of subjects all without hearing defects. These changes which have never before been reported, confirm multisystem involvement in this disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Acoustic Stimulation
  • Adolescent
  • Adult
  • Aged
  • Evoked Potentials, Auditory*
  • Evoked Potentials, Visual
  • Female
  • Hereditary Sensory and Motor Neuropathy / physiopathology*
  • Humans
  • Male
  • Middle Aged
  • Optic Atrophies, Hereditary / complications
  • Optic Atrophies, Hereditary / genetics
  • Optic Atrophies, Hereditary / physiopathology*
  • Reaction Time
  • Vestibulocochlear Nerve Diseases / physiopathology*