[Optic neuropathy in Strumpell-Lorrain disease: presentation of a clinical case and literature review]

Bull Soc Belge Ophtalmol. 2002;(286):9-14.
[Article in French]

Abstract

This report describes a 36-year-old female with bilateral optic atrophy. Physical examination was normal except for subclinical signs of lower limbs spasticity. Her father was affected of Strumpell-Lorrain disease. The clinical characteristics of Hereditary Spastic Paraplegia or Strumpell-Lorrain disease are: progressive spasticity of the lower limbs and pathological reflexes. Pathologically, this disease is characterized by a degeneration or demyelinization of the cortico-spinal system and, to a lesser extent, of the posterior cord and the spino-cerebellar system. Optic neuropathy and dyschromatopsy have already been reported in a small group of patients. Visual acuity ranged from 20/20 to 20/200. Visual field alterations consisted of superior bitemporal amputation. Fundus examination showed optic atrophy. The incidence of optic neuropathy in Strumpell-Lorrain disease is quite high but its presence is often overlooked. Most patients are asymptomatic, but signs of optic nerve dysfunction are evident at clinical examination. According to some authors, there is an inverse relationship between the impairment of walking and the visual dysfunction. This was also noted in our case.

Publication types

  • Case Reports
  • English Abstract
  • Review

MeSH terms

  • Adult
  • Demyelinating Diseases / genetics
  • Female
  • Humans
  • Optic Atrophy / genetics*
  • Paraplegia / diagnosis*
  • Paraplegia / genetics*
  • Pyramidal Tracts
  • Syndrome