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Case Reports
. 2021 May-Jun;24(3):405-409.
doi: 10.4103/aian.AIAN_405_20. Epub 2020 Jul 31.

Vogt-Koyanagi-Harada Syndrome - A Neurologist's Perspective

Sumanth Shivaram  1 , Madhu Nagappa  1 , Doniparthi V Seshagiri  1 , Jayanth Shimoga Shanthakumar  1 , Swayang Sudha Panda  1 , Ravi Anadure  1 , B N Nandeesh  2 , Yasha T Chickabasaviah  2 , Rose D Bharath  3 , Joy Vijayan  1 , Bakula Kashyap  4 , Sanjib Sinha  1 , Arun B Taly  1
Affiliations
Case Reports

Vogt-Koyanagi-Harada Syndrome - A Neurologist's Perspective

Sumanth Shivaram et al. Ann Indian Acad Neurol. 2021 May-Jun.

Abstract

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.

Keywords: Aseptic meningitis; Vogt-Koyanagi-Harada syndrome; brain-eye-ear (BEE) syndromes; exudative retinal detachment; melanin-laden macrophages; poliosis.

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Conflict of interest statement

There are no conflicts of interest.

Figures

Figure 1
Figure 1
Axial T1-weighted and post-contrast T1-weighted sections of the orbit in patient 1 show choroid enhancement (a and b, white arrows). Histopathology of skin in VKH: Normal skin (c); axillary skin biopsy in patient 1 showing melanin depletion (d, black arrows); melanophagocytosis in dermal macrophages (e, black arrows)
Figure 2
Figure 2
Shows loss of melanin pigment in a few eyelashes suggesting poliosis (a, arrows), optic disc hyperemia in fundus photograph (b, arrow), choroid thickening on B-scan of the orbit (c, arrow), abnormal optic disc staining (d, arrow), and leakage of dye in fundus fluorescein angiogram (d, asterisks) and melanin laden macrophages in cerebrospinal fluid (e and f, arrows) in patient 2
See this image and copyright information in PMC

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