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Case report: patient, 24 year, with Vogt Koyanagi Harada (VKH) incomplete syndrome and Radiologically isolated syndrome (RIS)

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Case report: Female, 24 years old, previously healthy, presented in december 2021 an acute posterior uveitis associated with serous retinal detachment , tinnitus and vertigo. Ophthalmological evaluation was performed with suspected Vogt Koyanagui Harada (VKH) syndrome, treatment with intravenous methylprednisolone was initially with partial response. It remained without maintenance treatment. Two month later she was with worse ocular complaints, binocular involvement, that didn't get better after oral corticoid administration. Magnetic resonance imaging (MRI) orbits showed thickening and enhancement of the posterior contour of the right eyeball (suggestive of VKH) and brain MRI exhibit hypersignal areas in white matter mainly periventricular as well as infratentorial in FLAIR and T2 sequences and post-gadolinium enhancement of two lesion (compliant with criteria for Radiologically isolated syndrome (RIS)). No oligoclonal bands were detected. The patient had no cutaneos lesions.
Discussion: VKH syndrome is a chronic disorder characterized by inflammation of the uvea, meninges, auditory system, and integumentary system. It may present clinical and/or radiological that can mimic autoimmune demyelinating diseases of the central nervous system (CNS) such as Multiple Sclerosis (MS). Briefly the revised diagnostic criteria for VKH include: 1-absence of penetrating ocular trauma; 2-absence of evidence of concomitant eye disease; 3-bilateral uveitis; 4- neurological and auditory manifestation; 5- cutaneous alteration. We described a case of incomplete VKH disease (based on diagnostic criteria) in which the brain MRI demonstrated demyelinating white matter abnormalities compatible with the revised 2017 McDonald Criteria for dissemination in space. In the absence of clinical demyelinating episodes it was classified as a RIS. Besides the treatment and prognosis for both conditions are different, the early and accurate diagnosis is very important to avoid complications of delayed diagnosis or unnecessary medications. However, differential diagnosis of the combination of uveitis and CNS demyelination may include a systemic or CNS vasculitis, systemic lupus erythematosus , Behcet’s disease, sarcoidosis, and paraneoplastic disorders. More studies in the area need to be carried out.
Final comments:A rare case report of incomplete VKH disease in which the brain MRI demonstrated demyelinating white matter abnormalities compatible with RIS.