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Título

VOUGT-KOYANAGI-HARADA DISEASE, a rare etiology of sterile meningitis - a case report

RESUMO

CASE REPORT: A 16 year old Venezuelan boy with no previous comorbidities, who was admitted at the emergency room presenting headache associated with bilateral conjunctival hyperemia and reduced visual acuity, limited to light perception. The signs and symptoms presented have been developing during the course of 14 days. There were no other neurological findings. A head computed tomography scan accused a diffuse enlargement of the ventricular system, suggesting hydrocephalus, and there was an altered signal inside both orbits compatible with retinal detachment. In addition, the analysis of the cerebrospinal fluid revealed pleocytosis, with a monocytes predominance. The patient underwent neurosurgery and a ventriculoperitoneal shunt had been installed. The ophthalmologic evaluation pointed out a serous panuveitis. The hypothesis of Vogt–Koyanagi–Harada disease has been raised and the patient was treated with a 5-day course of methylprednisolone followed by oral glucocorticoid and ophthalmological follow-up. DISCUSSION: Vogt–Koyanagi–Harada (VKH) disease is an important and rare cause of sterile meningitis. It is a systemic autoimmune disorder that target pigmented tissues of the body, affecting particularly the eyes, but also the skin, meninges and inner ear either, resulting in neurological symptoms such as headache, cranial nerves palsy and tinnitus. The prevalence of VKH varies in different populations in the world, accounting for 2 to 4% of all uveitis in Brazil. The diagnosis is based on clinical findings and must follow the criteria proposed by the American Uveitis Society, including bilateral ocular involvement with no history of penetrating ocular trauma or surgery, along with neurological or integumentary findings. The goal of treatment is to suppress intraocular inflammation and prevent potential visual impairment, therefore early diagnosis and treatment are essential. FINAL COMMENTS: It’s important sharing cases like this since it is a relatively rare diagnosis of sterile meningitis in clinical practice, hence often underdiagnosed. It is vital that physicians pay attention on the ophthalmological manifestations associated since they are the most frequent ones and the base of the diagnostic criteria. Once more, it is of utmost importance the fast diagnosis followed by and early and aggressive treatment with systemic corticosteroid to reduce subsequent recurrences and avoid complete and irreversible visual loss.

Palavras Chave

VOUGT-KOYANAGI-HARADA DISEASE; sterile meningitis; headache