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Título

Flair-hyperintense Lesions In Anti-MOG-Associated Encephalitis With Seizures (Flames): A Misunderstood Entity With Diagnostic Limitations

RESUMO

A 73-year-old male was admitted in a tertiary hospital due to progressive weakness in the right arm and later in the right leg, over three days, along with dysarthria and temporospatial disorientation. Was initially approached as an acute ischemic stroke with associated dementia, and, after hospital discharge, about one week from the start of the symptoms, he started to present focal aware clonic seizures in the right side. At admission, he was globally aphasic, with frontal release signs and right spastic hemiplegia. Brain magnetic resonance imaging (MRI) did not reveal ischemic stroke signs, but retrospective analysis of previous MRI showed left parieto-occipital cortical and bilateral insula hyperintensities on fluid attenuated inversion recovery (FLAIR) with cortical post-gadolinium enhancement, with notable progression. CSF analysis did not show inflammatory markers and Multiplex PCR was negative. Due to the symptoms progression and radiologic features, an hypothesis of FLAIR hyperintense cortical lesions in MOG associated Encephalitis with Seizures (FLAMES) was made. The patient was then treated with high dose intravenous steroids and albumin plasmapheresis, with an excellent response, being discharged after two weeks speaking and walking without help. After one month, the patient is taking azathioprine and on corticosteroids tapering, still able to walk independently and speak with only a small latency of speech response.
Myelin oligodendrocyte glycoprotein (MOG) antibody associated disease (MOGAD) is an inflammatory affliction of the central nervous system (CNS), and similar cases were described with FLAMES (4). Clinical features include seizures, headache, fever and cortical symptoms referable to the FLAMES location, and 95% reported cases at least two of these four findings (5). Although initially described as a unilateral cortical encephalitis, bilateral cortical involvement and possible meningeal inflammation could indicate a broader disease spectrum. The syndrome is highly steroid-responsive (1).
FLAMES is a rare condition that remains underdiagnosed. Although, we still have a lot of limitations to diagnoses, considering the unavailability and high prices of the tests. Recognition of this distinct clinico-radiographic syndrome may facilitate prompt diagnosis and treatment. Therefore, the reported cases suggest excellent response to adequate treatment.

Palavras Chave

MOGAD
FLAMES
Encephalitis