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Título

Vogt-Koyanagi-Harada syndrome, an important diferencial diagnosis

RESUMO

Case presentation: Female, 44 years old attended at Neurology service with 15 days of bitemporal, throbbing headache, intensity 8/10, associated with nausea, vomiting, hearing loss (HL), low visual acuity (VA) and pain on eye movement in the left eye (LE). After 7 days, she evolved with low VA in the right eye (RE), with loss of color saturation and presence of central stochoma. She reported a history of previous, mild, perimenstrual headache and no ophthalmologic complaints. Denied other symptoms, trauma, use of medications, allergies or hospitalizations. On examination: preserved eye movements. VA of the RE: counting fingers (CF) 50 cm. VA of LE: CF 30 cm. Reduced photomotor reflex bilaterally, and biomicroscopy with thin keratic precipitates and posterior uveitis (2+/4). Bilateral optic disc edema, with vascular tortuosity and bilateral serous retinal detachment. Further investigation showed: lumbar puncture with opening pressure 23 cmH2O (and 1 leukocyte, non-reactive VDRL, total proteins 25); Normal magnetic resonance imaging (MRI); Mixed HL audiometry and orbital magnetic resonance imaging with signs of chorioretinitis. In addition to negative serological tests and negative antiaquaporin 4 research. Discussion:
Vogt-Koyanagi-Harada Syndrome (VKHS) is a systemic autoimmune disease that mainly affects melanocyte-rich structures such as the retina, inner ear and central nervous system. It contains 4 distinct clinical stages: prodromal, uveitic, chronic and recurrent. The previously healthy patient sought care due to unilateral eye pain, which developed bilaterally after 7 days, associated with other ocular symptoms and HL. MRI rule out tumors or signs of increased intracranial pressure and demyelinating diseases. Addicionaly, acute worsen of HL, along with serous retinal detachments bilaterally and posterior uveitis. And a negative viral serology and anti-aquaporin 4 narrows criteria for VKHS. Treatment with 5 days of 1g methylprednisolone intravenous was made with significant outcome, showed by improvement of VA and retinal detachments at OCT, along with no more headache episodes. Final comments:
As a rare syndrome, the diagnosis of VKHS requires a complete anamnesis and a good physical examination, associated with complementary tests, such as OCT and serological tests, to aid diagnosis.

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Área

Neuroimunologia