Open AccessVogt-koyanagi-harada syndrome - A neurologist's perspective
Author(s) -
Sumanth Shivaram,
Madhu Nagappa,
Doniparthi V. Seshagiri,
Jayanth Shimoga Shanthakumar,
Swayang Sudha Panda,
Ravi Anadure,
B N Nandeesh,
Yasha T. Chickabasaviah,
Rose Dawn Bharath,
Joy Vijayan,
Bakula Kashyap,
Sanjib Sinha,
Arun B. Taly
Publication year - 2021
Publication title -
annals of indian academy of neurology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.427
H-Index - 31
eISSN - 1998-3549
pISSN - 0972-2327
DOI - 10.4103/aian.aian_405_20
Subject(s) - medicine , vogt–koyanagi–harada disease , pleocytosis , cerebrospinal fluid , meningitis , sarcoidosis , pathology , aseptic meningitis , brain biopsy , choroid , fundus (uterus) , lymphocytic pleocytosis , biopsy , ophthalmology , encephalitis , retina , disease , surgery , immunology , virus , physics , optics
Vogt-Koyanagi-Harada (VKH) syndrome is an immune-mediated granulomatous disease which affects melanin-rich organs like eyes, skin, nervous system, and ears. Neurological and auditory manifestations usually precede the involvement of other sites. Patients may manifest with "complete" or "incomplete" syndrome. We report two patients who presented with acute headache and impaired vision. Fundus examination revealed optic disc hyperemia and exudative retinal detachment which provided a clue for the diagnosis at the bedside. Fundus fluorescein angiogram (FFA) revealed abnormal dye leakage, whereas B scan showed choroid thickening. Cerebrospinal fluid (CSF) pleocytosis contrasted with unremarkable brain magnetic resonance imaging and lack of meningeal signs. Melanophagocytosis was evidenced by melanin-laden macrophages in CSF and skin biopsy. This finding is specific for VKH syndrome and helps to clinch the diagnosis even when the complete syndrome is not present cross-sectionally. VKH syndrome should be suspected in patients with aseptic meningitis if tests for common infectious and immune-mediated diseases are negative.