Basilar leptomeningitis in Vogt-Koyanagi-Harada disease

Abstract

A 35-year-old Filipino man had acute visual loss, tinnitus, dysacusis, and severe horizontal vertigo. Examination revealed bidirectional gaze-evoked nystagmus and panuveitis with bilateral inferior serous retinal detachments (figure 1) characteristic of Vogt-Koyanagi-Harada disease.1 Brain MRI demonstrated mesencephalic, pontine, and cerebellar leptomeningeal enhancement (figure 2). There was cerebrospinal lymphocytic pleocytosis. Following IV Solu-Medrol, his fundus was significantly improved.Vogt-Koyanagi-Harada disease is a uveomeningeal syndrome of panuveitis with bullous serous retinal detachment, meningitis, and dysacusis.1 Although the targets of inflammation, melanin-containing cells, 1,2 are predominantly located over the ventral medulla, 3 our patient demonstrated a ponto-mesencephalic and cerebellar predilection of inflammation.

Vogt-Koyanagi-Harada syndrome: A novel case and brief review of focal neurologic presentations

“… 3 Focal parenchymal lesions with associated neurologic deficits have been reported in relatively few VKH cases. 4 – 6 One case presented with Wallenberg syndrome 4 and another with brainstem encephalitis and multiple cranial nerve palsies. 5 It is interesting that our patient had the highest disease burden in the CPA, given the higher concentration of melanocytes over the ventrolateral medulla in humans.…”

“… 7 In a recently published case, a predilection for pontomesencephalic and cerebellar areas was also noted. 6 …”