Dados do Trabalho

Título

A FLAMES-LIKE PRESENTATION IN A YOUNG CHILD: AN UNCOMMON MOG ASSOCIATED DISEASE PHENOTYPE

Apresentação do caso

A previously healthy 4-year-old bolivian girl presented to our hospital with right-sided weakness and ipsilateral ataxia, preceded by 3 days of acute headache and mild lethargy. Two weeks before she had had flu-like symptoms. Parents denied seizures or fever. Brain MRI with gadolinium showed left superior frontal gyrus and perirolandic area hyperintensity on T2-FLAIR, without overlying leptomeningeal enhancement. Electroencephalography showed anterior left frontal disorganization without epileptiform activity. Serologies were negative. Lumbar puncture revealed normal parameters, including no CSF-specific oligoclonal bands (OCB). With suspicion of a possible demyelinating event, MOG-IgG was tested and came positive. Intravenous methylprednisolone 30 mg/kg daily was started for 5 days, with complete clinical recovery. In the last follow-up she was asymptomatic, without immunotherapy of maintenance and 6-month-follow up brain MRI disclosed only a subtle left frontal cortical hyperintensity.

Discussão

Unilateral cortical FLAIR-hyperintense lesions anti-MOG-associated encephalitis with seizures (FLAMES) is a rare anti-MOG phenotype first described in 2017 and observed in young adult patients. Key clinical features include seizures (85%), headache (70%), fever (65%) and cortical symptoms referable to the FLAIR hyperintense location (55%) and 95% subjects satisfied at least two findings, like our patient, although she remained seizure-free. At present, none of the reviewed patients in literature with anti-MOG antibody negative presented FLAMES. CSF usually discloses pleocytosis and negative OCB. Although initially described as a unilateral cortical encephalitis, bilateral cortical involvement and possible meningeal inflammation cited may reflect a broader disease spectrum. The syndrome is highly steroid-responsive and the majority of cases showed a full recovery with IVMP, so early identification will lead to prompt treatment.

Comentários finais

We described a case similar to FLAMES, a rare presentation of anti-MOG disease. Improved awareness about it is fundamental to guarantee correct diagnosis and institution of immunotherapy, particularly in more severe cases.

Referências (se houver)

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Jain K, Cherian A, K P D, P R, Thomas B, J N. "FLAMES: A novel burning entity in MOG IgG associated disease". Mult Scler Relat Disord. 2021 Apr;49:102759. doi:10.1016/j.msard.2021.102759. Epub 2021 Jan 17. PMID: 33508569.

Budhram A, Sechi E, Nguyen A, Lopez-Chiriboga AS, Flanagan EP. FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis With Seizures (FLAMES): Is immunotherapy always needed to put out the fire? Mult Scler Relat Disord. 2020 Sep;44:102283. doi:
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Budhram A, Mirian A, Le C, Hosseini-Moghaddam SM, Sharma M, Nicolle MW. Unilateral cortical FLAIR-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures (FLAMES): characterization of a distinct clinico-radiographic syndrome. J Neurol. 2019
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Andrini G, Asioli GM, Spinardi L, D'Angelo R. FLAMES over an "extinguished fire": a MOG-associated disorder case report. Neurol Sci. 2023 Mar;44(3):1131-1134. doi:10.1007/s10072-022-06576-w. Epub 2022 Dec 27. PMID: 36574177

Palavras Chave

MOG, focal encephalitis, seizures.

Declaração de conflito de interesses de TODOS os autores

Renata Barbosa Paolilo received support for participating in scientific meetings from Biogen,
Merck and Roche; and received speaking honoraria from Novartis.
The other authors have no conflict of interest.