Ophthalmological changes in hereditary spastic paraplegia and other genetic diseases with spastic paraplegia

J Neurol Sci. 2020 Feb 15:409:116620. doi: 10.1016/j.jns.2019.116620. Epub 2019 Dec 6.

Abstract

Ophthalmological abnormalities may occur in specific subtypes of hereditary spastic paraplegia (HSP) and in genetic diseases that present with spastic paraplegia mimicking HSP. These ophthalmological changes may precede the motor symptoms and include pigmentary retinal degeneration, ophthalmoplegia, optic atrophy, cataracts and nystagmus. Some ophthalmological abnormalities are more prevalent in specific forms of HSP. Considering that the diagnosis of HSP is usually difficult and complex, specific ophthalmological changes may guide the genetic testing. There are other genetic diseases such as autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), X-linked adrenoleukodystrophy and spastic paraplegia, optic atrophy and neuropathy (SPOAN) that may mimic HSP and also may present with specific ophthalmological changes. In this article, we review the main ophthalmological changes observed in patients with HSP and HSP-like disorders.

Keywords: Hereditary spastic paraplegia; Ophthalmological changes; Optic atrophy; Spastic paraplegia.

Publication types

  • Review

MeSH terms

  • Diagnostic Techniques, Ophthalmological*
  • Eye Diseases / diagnostic imaging*
  • Eye Diseases / epidemiology
  • Eye Diseases / genetics*
  • Humans
  • Muscle Spasticity / diagnostic imaging
  • Muscle Spasticity / epidemiology
  • Muscle Spasticity / genetics
  • Optic Atrophy / diagnostic imaging
  • Optic Atrophy / epidemiology
  • Optic Atrophy / genetics
  • Paraplegia / diagnostic imaging
  • Paraplegia / epidemiology
  • Paraplegia / genetics
  • Spastic Paraplegia, Hereditary / diagnostic imaging*
  • Spastic Paraplegia, Hereditary / epidemiology
  • Spastic Paraplegia, Hereditary / genetics*
  • Spinocerebellar Ataxias / congenital
  • Spinocerebellar Ataxias / diagnostic imaging
  • Spinocerebellar Ataxias / epidemiology
  • Spinocerebellar Ataxias / genetics

Supplementary concepts

  • Spastic ataxia Charlevoix-Saguenay type