Teaching neuroimages: cerebral white matter involvement in a patient with Vogt-Koyanagi-Harada syndrome

Neurology. 2013 Sep 10;81(11):e85-6. doi: 10.1212/WNL.0b013e3182a43b01.

Abstract

A 27-year-old man presented with decreased vision bilaterally. Ophthalmologic examination revealed panuveitis with bilateral serous retinal detachments (figure 1) characteristic of Vogt-Koyanagi-Harada (VKH) syndrome. Analysis of CSF revealed mild pleocytosis. No oligoclonal bands were detected. On MRI, there was bilateral abnormal thickening of the choroid with retinal detachment (figure 2, A and B), with hyperintense lesion on fluid-attenuated inversion recovery (FLAIR) image in the periventricular deep white matter (figure 2C). VKH syndrome is one of the causes of uveo-meningeal syndromes. Differential diagnosis of VKH syndrome includes Wegener granulomatosis, sarcoidosis, and Behçet disease.(1) The main disorders showing association with hyperintense plaques on deep white matter FLAIR images are inflammatory, metabolic, vascular, degenerative, autoimmune, and demyelinating diseases. MRI is a helpful tool in diagnosing VKH syndrome; in addition to the typical bilateral ocular findings, scattered periventricular white matter lesions on FLAIR are also described.(2.)

Publication types

  • Case Reports
  • Editorial

MeSH terms

  • Adult
  • Cerebral Cortex / pathology*
  • Diagnosis, Differential
  • Fluorescein Angiography
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Nerve Fibers, Myelinated / pathology*
  • Retinal Detachment / diagnosis
  • Retinal Detachment / etiology
  • Tomography, Optical Coherence
  • Uveomeningoencephalitic Syndrome / cerebrospinal fluid
  • Uveomeningoencephalitic Syndrome / pathology*