Dados do Trabalho
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.
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.
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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MOG, focal encephalitis, seizures.
Fonte de Fomento (se houver)
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.
Neuroimunologia, esclerose múltipla e outras doenças desmielinizantes
Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo - HCFMUSP - São Paulo - Brasil
Ana Beatriz Arruda Carvalho de Oliveira, Cristiani Rocha Lima Cruz, Larissa Baccoli de Souza, Flora Cruz de Almeida Ximenes, Daniel Shoji Hayashi, Renata Silva de Mendonça, Murillo Bruno Braz Barbosa, Daiane Maryane Cardoso Santos, Renata Barbosa Paolilo