Neuro-ophthalmology of subacute sclerosing panencephalitis: two cases and a review of the literature

Curr Opin Ophthalmol. 2012 Nov;23(6):466-71. doi: 10.1097/ICU.0b013e328358b196.

Abstract

Purpose of review: To review the literature on early visual manifestations of subacute sclerosing panencephalitis (SSPE) with regard to two patients who had visual problems preceding the onset of neurological symptoms. One patient had cortical visual disturbances and the other had visual loss due to retinal pigment epithelial changes.

Recent findings: SSPE is a chronic encephalitis characterized by a history of measles infection and a progressive disease of the central nervous system that still occurs frequently in countries with insufficient measles immunization. Visual manifestations can occur as a result of involvement of the pathways that lead from the retina to the occipital cortex during the course of the disease, but are rare as a presenting sign. Fundus changes, especially macular retinitis and macular pigment disturbances, appear to be the most common ocular manifestations of SSPE.

Summary: Ophthalmologists must be aware that SSPE can knock their door with ocular findings of SSPE, months or years before the onset of neurological symptoms.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Adolescent
  • Amantadine / therapeutic use
  • Antibodies, Viral / blood
  • Antibodies, Viral / cerebrospinal fluid
  • Anticonvulsants / therapeutic use
  • Antiviral Agents / therapeutic use
  • Blindness / diagnosis
  • Blindness / drug therapy
  • Blindness / etiology*
  • Carbamazepine / therapeutic use
  • Drug Therapy, Combination
  • Electroencephalography
  • Fatal Outcome
  • Female
  • Headache / etiology
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Inosine Pranobex / therapeutic use
  • Magnetic Resonance Imaging
  • Male
  • Measles virus
  • Nausea / etiology
  • Subacute Sclerosing Panencephalitis / complications*
  • Subacute Sclerosing Panencephalitis / diagnosis
  • Subacute Sclerosing Panencephalitis / drug therapy

Substances

  • Antibodies, Viral
  • Anticonvulsants
  • Antiviral Agents
  • Immunoglobulins, Intravenous
  • Carbamazepine
  • Amantadine
  • Inosine Pranobex