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Vogt-Koyanagi-Harada syndrome (VKHS) is a multisystem disorder that typically presents with bilateral uveitis, often associated with neurological and/or audiological features. However, it can present with neurological features before the ocular changes become apparent. VKHS is well known to ophthalmologists: though less common in Caucasians, it can be responsible for up to 20% of presentations with acute uveitis in certain racial groups.1 2 Neurologists rarely encounter it, meaning that they can be caught off guard, as in the case of acute meningitis reported by Street and colleagues in this issue of Practical Neurology.3 Early diagnosis is important as this allows appropriate early treatment which in turn is associated with a better visual outcome.4 5 This article will provide a brief overview but the interested reader is referred to recent excellent reviews for more information.1 2 6
Historical background
VKHS was probably described as early as the 12th century7 but the first ‘modern’ description was in 1906 by Alfred Vogt, a Swiss ophthalmologist who described a 16-year-old with iridocyclitis and poliosis of the eyelashes.8 Two Japanese ophthalmologists, Yoshizo Koyanagi and Einosuke Harada, subsequently published case series in 1929 and 1926, respectively.9 10 The term ‘Vogt-Koyanagi disease’ was coined in 193911 and was used in parallel with ‘Harada’s disease’12 until the modern term, Vogt-Koyanagi-Harada syndrome was introduced in about 1955.7
Clinical background
VKHS is an autoimmune, granulomatous, inflammatory disorder mediated by CD4+T cells that target melanocytic antigens, probably the enzyme tyrosinase. These antigens are most widely found in eye, central nervous system, inner ear and skin. The trigger is unknown but a viral cause has been postulated, and there does appear to be a genetic influence, with increased susceptibility in people with certain HLA haplotypes, particularly HLA DRB1*0405 and HLA-DQ4.2 13 Ocular histology shows a …
Footnotes
Contributors CJL is the sole author of this manuscript.
Funding The author has not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned. Internally peer reviewed.
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- Editors’ commentary
- Neurological rarities
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