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Título

Young patient diagnosed with CRION with visual improvement after use of human immunoglobulin: a case report.

RESUMO

Case report: A 26-year-old female patient with low visual acuity and photophobia in the left eye. The condition evolved progressively, with the appearance of pain on ocular mobilization 2 days after the onset of symptoms. Condition occurred acutely, already waking up with visual changes. The patient had several similar episodes in both eyes since the age of 14, with a milder condition, with no limitation of daily activities in most episodes. The current episode it is the second with an impact on daily activities. In the first severe episode, she underwent pulse therapy with methylprednisonole and her symptoms completely improved. In an ophthalmological evaluation, an important change in the visual field of the left eye was observed by the campimetry. MRI of the brain was performed, with a slight hypersignal on T2 in the left optic nerve. Cerebrospinal fluid was normal Requested rheumatological tests, anti aquaporin 4, and anti MOG research, all negative. The patient was again submitted to pulse therapy with methylprednisolone, without a satisfactory clinical response. Next step was intravenous immunoglobulin infusion, and a significant positive response of improvement in the visual acuity was observed. In this context, in view of the clinical manifestation and the complementary exams, the diagnosis of Chronic Relapsing Inflammatory Optic Neuropathy (CRION) was made.

Discussion: CRION is a rare form of autoimmune optic neuropathy. Is characterized by vision loss and pain, and the occurrence of symptoms for more than one episode. MRI shows contrast enhancement in the optic nerve, and a good response to corticosteroid therapy is expected. Absence or suspension of treatment can lead to recurrence of symptoms, with possible irreversible visual loss. An atypical aspect of the case described is the therapeutic response only with the use of human Immunoglobulin. Kalish at el. described a series of CRION cases with a favorable outcome in selected cases treated with venous immunoglobulin.
Final coments: Patients with CRION are a diagnostic challenge. It is important for neurologists to maintain clinical suspicion. Initiation of early appropriate therapy may promote a better clinical response and a better functional outcome. The treatment of acute CRION events with human immunoglobulin is not the first therapeutic option, but as described in the literature, it becomes a viable option, especially in cases of first-line refractoriness.

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Área

Neuroimunologia