Vogt-Koyanagi-Harada syndrome is an inflammatory condition in which ocular inflammation or uveitis is the most significant feature of the illness. Neurologists may be called on to diagnose the prodromal phase of the disease in which the predominant symptoms are headache, meningeal signs, CSF pleocytosis, and, less often, focal neurologic findings. In this update, the authors cite work that shows that earlier initiation of either glucocorticoids or immunomodulatory measures significantly reduces late visual morbidity in patients with Vogt-Koyanagi-Harada syndrome and that the neurologic manifestations can include cerebral ischemia in the adult and pediatric population.
• Vogt-Koyanagi-Harada syndrome is primarily an ocular inflammatory disease, but the prodrome often includes signs and symptoms that bring the patient to a neurologist, including headache, meningeal signs, and CSF pleocytosis.
• Melanin pigment-containing cells are often involved, including the retinal pigment epithelium in the eye producing “sunset glow” fundus appearance and cutaneous melanocytes producing vitiligo and poliosis.
• The disease is encountered more frequently in persons of Asian, Latin, and Mediterranean descent than in other ethnic groups.
Historical note and terminology
The subject of this review is a clinical syndrome in which pigmentary alterations in various tissues are associated with ocular and meningeal inflammation. The combination of poliosis (the graying, or whitening, of a shock of scalp hairs, from the Greek for "gray"), and ocular inflammation was noted by the Arab physician Ali Ibn Isa (Ali ben Issa), who died in 1010 (Pattison 1965; Moorthy et al 1995), and by others including Vogt (Vogt 1906). Both Vogt and Koyanagi described cases that included the combination of primarily anterior uveitis (bilateral iridocyclitis) with extraocular manifestations, including pigment loss from skin (vitiligo), hair, and lashes (poliosis); hair loss (alopecia); and hearing loss with tinnitus (Vogt 1906; Koyanagi 1929). Koyanagi's 6 cases were described in 1929, but in 1926, Harada had described similar extraocular manifestations with a primarily posterior uveitis, characterized by exudative retinal detachments and pleocytosis in the cerebrospinal fluid (Harada 1926). Moorthy and colleagues credit Babel (Babel 1932), and Bruno and McPherson (Bruno and McPherson 1949) with recognizing the essential unity of the cases described by Vogt, Koyanagi, and Harada (Moorthy et al 1995). Herbort and Mochizuki reviewed the historical development of the syndrome emphasizing that both Koyanagi and Harada perceived the inherent unity of cases with seemingly disparate multisystem signs and symptoms, whereas Vogt was lucky enough to describe the first case extremely well (Herbort and Mochizuki 2007).
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