FLAMES (FLAIR Hyperintense Lesions in Anti-Myelin Oligodendrocyte Glycoprotein-associated Encephalitis with Seizures), a case report.

Ángeles Schteinschnaider , María Gala González, Romina Nélida Dominguez, Paulina Yañez, Mario Massaro


OBJECTIVE: Describe a pediatric patient with diagnosis of FLAMES.

CASE REPORT: A 9-year-old previously healthy boy, presented with acute gastroenteritis, fever, headache, generalized tonic-clonic seizures, absences, and acute sensory impairment. Physical examination did not show motor focus. Empirical treatment with antibiotics and antivirals was indicated. CSF results from lumbar puncture were normal. Brain magnetic resonance imaging showed multiple hyperintense areas on FLAIR/T2 sequences of bi-hemispheric asymmetric cortical involvement, without contrast enhancement. Spinal cord MRI was normal. Electroencephalogram (EEG) revealed slight disorganization of baseline rhythmicity with generalized, medium amplitude wave-paroxysms. Anti-MOG antibodies were detected in serum. Patient received five pulses of methylprednisolone with posterior tapering, and monthly gamma-globulin for five months. Due to seizure persistence, treatment with Rituximab was indicated with good response. At present, the patient is under treatment with valproic acid, levetiracetam and clobazam. Images remain stable.

CONCLUSION: FLAMES is a new entity which should be considered in patients presenting encephalitis, seizures, and characteristic lesions on FLAIR sequences, together with serum anti-MOG antibodies. Few cases have been reported in the literature, some with difficult-to-control epilepsy. Current treatment is based on clinical presentation. Further research is needed to establish guidelines ensuring appropriate management for these unusual cases.

Keywords: MOG - Encephalitis - MRI - FLAIR - Seizures

Ángeles Schteinschnaider
Raúl Carrera Institute for Neurological Research - FLENI
Argentina

María Gala González
Raúl Carrera Institute for Neurological Research - FLENI
Argentina

Romina Nélida Dominguez
Raúl Carrera Institute for Neurological Research - FLENI
Argentina

Paulina Yañez
Raúl Carrera Institute for Neurological Research - FLENI
Argentina

Mario Massaro
Raúl Carrera Institute for Neurological Research - FLENI
Argentina
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Ángeles Schteinschnaider 
Raúl Carrera Institute for Neurological Research - FLENI Argentina