Vogt-koyanagi-harada syndrome - A neurologist's perspective

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”...

Full description

Bibliographic Details
Main Authors: Sumanth Shivaram, Madhu Nagappa, Doniparthi V Seshagiri, Jayanth Shimoga Shanthakumar, Swayang Sudha Panda, Ravi Anadure, B N Nandeesh, Yasha T Chickabasaviah, Rose D Bharath, Joy Vijayan, Bakula Kashyap, Sanjib Sinha, Arun B Taly
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2021-01-01
Series:Annals of Indian Academy of Neurology
Subjects:
Online Access:http://www.annalsofian.org/article.asp?issn=0972-2327;year=2021;volume=24;issue=3;spage=405;epage=409;aulast=Shivaram
_version_ 1818891133828202496
author Sumanth Shivaram
Madhu Nagappa
Doniparthi V Seshagiri
Jayanth Shimoga Shanthakumar
Swayang Sudha Panda
Ravi Anadure
B N Nandeesh
Yasha T Chickabasaviah
Rose D Bharath
Joy Vijayan
Bakula Kashyap
Sanjib Sinha
Arun B Taly
author_facet Sumanth Shivaram
Madhu Nagappa
Doniparthi V Seshagiri
Jayanth Shimoga Shanthakumar
Swayang Sudha Panda
Ravi Anadure
B N Nandeesh
Yasha T Chickabasaviah
Rose D Bharath
Joy Vijayan
Bakula Kashyap
Sanjib Sinha
Arun B Taly
author_sort Sumanth Shivaram
collection DOAJ
description 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.
first_indexed 2024-12-19T17:35:58Z
format Article
id doaj.art-d2f72c93ca914e779d06262c0f0c161d
institution Directory Open Access Journal
issn 0972-2327
1998-3549
language English
last_indexed 2024-12-19T17:35:58Z
publishDate 2021-01-01
publisher Wolters Kluwer Medknow Publications
record_format Article
series Annals of Indian Academy of Neurology
spelling doaj.art-d2f72c93ca914e779d06262c0f0c161d2022-12-21T20:12:20ZengWolters Kluwer Medknow PublicationsAnnals of Indian Academy of Neurology0972-23271998-35492021-01-0124340540910.4103/aian.AIAN_405_20Vogt-koyanagi-harada syndrome - A neurologist's perspectiveSumanth ShivaramMadhu NagappaDoniparthi V SeshagiriJayanth Shimoga ShanthakumarSwayang Sudha PandaRavi AnadureB N NandeeshYasha T ChickabasaviahRose D BharathJoy VijayanBakula KashyapSanjib SinhaArun B TalyVogt-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.http://www.annalsofian.org/article.asp?issn=0972-2327;year=2021;volume=24;issue=3;spage=405;epage=409;aulast=Shivaramaseptic meningitisbrain-eye-ear (bee) syndromesexudative retinal detachmentmelanin-laden macrophagespoliosisvogt-koyanagi-harada syndrome
spellingShingle Sumanth Shivaram
Madhu Nagappa
Doniparthi V Seshagiri
Jayanth Shimoga Shanthakumar
Swayang Sudha Panda
Ravi Anadure
B N Nandeesh
Yasha T Chickabasaviah
Rose D Bharath
Joy Vijayan
Bakula Kashyap
Sanjib Sinha
Arun B Taly
Vogt-koyanagi-harada syndrome - A neurologist's perspective
Annals of Indian Academy of Neurology
aseptic meningitis
brain-eye-ear (bee) syndromes
exudative retinal detachment
melanin-laden macrophages
poliosis
vogt-koyanagi-harada syndrome
title Vogt-koyanagi-harada syndrome - A neurologist's perspective
title_full Vogt-koyanagi-harada syndrome - A neurologist's perspective
title_fullStr Vogt-koyanagi-harada syndrome - A neurologist's perspective
title_full_unstemmed Vogt-koyanagi-harada syndrome - A neurologist's perspective
title_short Vogt-koyanagi-harada syndrome - A neurologist's perspective
title_sort vogt koyanagi harada syndrome a neurologist s perspective
topic aseptic meningitis
brain-eye-ear (bee) syndromes
exudative retinal detachment
melanin-laden macrophages
poliosis
vogt-koyanagi-harada syndrome
url http://www.annalsofian.org/article.asp?issn=0972-2327;year=2021;volume=24;issue=3;spage=405;epage=409;aulast=Shivaram
work_keys_str_mv AT sumanthshivaram vogtkoyanagiharadasyndromeaneurologistsperspective
AT madhunagappa vogtkoyanagiharadasyndromeaneurologistsperspective
AT doniparthivseshagiri vogtkoyanagiharadasyndromeaneurologistsperspective
AT jayanthshimogashanthakumar vogtkoyanagiharadasyndromeaneurologistsperspective
AT swayangsudhapanda vogtkoyanagiharadasyndromeaneurologistsperspective
AT ravianadure vogtkoyanagiharadasyndromeaneurologistsperspective
AT bnnandeesh vogtkoyanagiharadasyndromeaneurologistsperspective
AT yashatchickabasaviah vogtkoyanagiharadasyndromeaneurologistsperspective
AT rosedbharath vogtkoyanagiharadasyndromeaneurologistsperspective
AT joyvijayan vogtkoyanagiharadasyndromeaneurologistsperspective
AT bakulakashyap vogtkoyanagiharadasyndromeaneurologistsperspective
AT sanjibsinha vogtkoyanagiharadasyndromeaneurologistsperspective
AT arunbtaly vogtkoyanagiharadasyndromeaneurologistsperspective