Vogt-Koyanagi-Harada disease presenting initially as aseptic meningoencephalitis

Eur J Neurol. 2000 Nov;7(6):719-22. doi: 10.1046/j.1468-1331.2000.00156.x.

Abstract

Vogt-Koyanagi-Harada (VKH) disease in a Gypsy woman was diagnosed 4 months after her initial complaints. The delay is explained by the facts that: (1) the characteristic ophthalmological symptoms, which usually herald the disease and ensure early diagnosis, developed only late during the course; and (2) only retrospective analysis of the cerebrospinal fluid (CSF) cell preparation proved the presence of melanin-laden macrophages (MLMs), specific for the syndrome. We emphasize that VKH syndrome may initially present as aseptic meningitis, without specific ophthalmological symptoms. In suspected cases a very detailed CSF cell analysis is needed, because the presence of MLMs could confirm the diagnosis. However, VKH syndrome has a much higher incidence in Asia; cases in other races, including white people in Europe, also occur.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Cyclosporine / therapeutic use
  • Cytoplasmic Granules / pathology
  • Diagnosis, Differential
  • Female
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Macrophages / pathology
  • Melanins / cerebrospinal fluid
  • Meningitis, Aseptic / diagnosis
  • Meningoencephalitis / diagnosis
  • Methylprednisolone / therapeutic use
  • Recurrence
  • Uveomeningoencephalitic Syndrome / cerebrospinal fluid
  • Uveomeningoencephalitic Syndrome / diagnosis*
  • Uveomeningoencephalitic Syndrome / drug therapy

Substances

  • Immunosuppressive Agents
  • Melanins
  • Cyclosporine
  • Methylprednisolone