Open AccessVogt-Koyanagi-Harada syndrome presenting with encephalopathy
Author(s) -
AlirezaE Naeini,
Dana Daneshmand,
Farzin Khorvash,
Ahmad Chitsaz
Publication year - 2014
Publication title -
annals of indian academy of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.427
H-Index - 31
eISSN - 1998-3549
pISSN - 0972-2327
DOI - 10.4103/0972-2327.128585
Subject(s) - medicine , presentation (obstetrics) , encephalopathy , vogt–koyanagi–harada disease , disease , pediatrics , blindness , dermatology , case presentation , pathology , surgery , optometry
Vogt-Koyanagi-Harada (VKH) is a rare syndrome affecting tissues containing melanocytes. The possibility of its autoimmune pathogenesis is supported by high frequent HLA-DR4 presentation, commonly associated with other autoimmune diseases. Eyes are the main affected organs, resulting in blindness. Brain disease is a late-onset event, and is extremely rare. Here, we are reporting a 57-year-old woman, a known case of VKH syndrome, presenting with brain encephalopathy several decades after the initial presentation. We think this long period between initial presentation and presentation of encephalopathy due to VKH syndrome has not been described before. She was treated with corticosteroids and discharged home with a good general condition.