Authors :
Presenting Author: Noushin Chini Foroush, MD – Western health, Victoria, Australia
Tissa Wijeratne, Professor of neurology – Head of neurology department, neurologist, Neurology department at Western health, Melbourne university
Rationale:
Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a rare type of CNS neuroinflammatory disorder that presents with optic neuritis or myelitis. FLAMES (Flair-hyperintense Lesions in Anti-MOG-associated Encephalitis with Seizures), first described in 2017, is a rare manifestation of MOGAD that presents with seizures, headache, and fever.
1 Misdiagnosis or delayed diagnosis of this phenotype of MOGAD is common and has been reported in 47% of presentations.
2 Methods:
A scoping review was conducted with Arksey et al methodology.
3 This case report was completed with the patient’s informed consent.
Results:
We report a unique manifestation of FLAMES. A 24-year-old male presented with a headache and fever. He had a normal brain MRI. He had raised serum white cell count with negative infective screening. His CSF showed pleocytosis with 184 lymphocytes. He was discharged with a diagnosis of aseptic meningitis. Seven months later, he presented with a right-sided focal to bilateral tonic-clonic seizure (FTBTC). He had a normal brain MRI. However, his EEG showed left temporal slowing. He was diagnosed with focal epilepsy and commenced on levetiracetam. The patient self-ceased the medication after being seizure free for one year. Three years later, he presented with a seizure and left-sided headache. His seizure was characterized as left-sided FTBTCs. His EEG testing and CSF analysis were reported normal. His brain MRI demonstrated right frontoparietal cortical FLAIR hyperintensities. His serum MOG antibodies were positive with a high 1:100 titre. He was diagnosed with MOGAD and commenced on oral steroids with full recovery. In our literature review, we summarized 38 cases of MOGAD with FLAMES and their seizure semiology (Table 1). A total of 67% (24/38) of patients had focal onset seizures, 45% (17/38) presented with focal motor and non-motor seizures and 26% (10 /38) with FTBTC. In 47% (18/38) of cases, generalized tonic-clonic seizures were described. Interictal EEG showed epileptiform discharges in 43% of cases, 23% focal slowing and one case (3%) had an electrographic seizure. 27% of cases had normal interictal EEG. Eight patients did not have an EEG as a part of their management. In three cases, there was no CSF result. Among subjects with a CSF investigation, 66% had lymphocytic patterns while the rest showed normal cell count. The delay in diagnosis varies between two weeks to five years, Majority of delayed diagnosis was initially diagnosed as infective focal meningoencephalitis.
Conclusions:
This literature review and our case highlight that focal onset seizures are more common in FLAMES and the triad of headache, fever, and seizures should raise suspicion of FLAMES to assist in early diagnosis.
References:
1 Budhram A, et al. Meningo-cortical manifestations of myelin oligodendrocyte glycoprotein antibody-associated disease: Review of a novel clinico-radiographic spectrum. Front Neurol. 2022 Oct 20;13:1044642.
2 Valencia-Sanchez C, et al. Cerebral Cortical Encephalitis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease. Ann Neurol. 2023 Feb;93(2):297-302.
3Arksey H, et al, coping studies: towards a methodological framework, 8:1, 19-32Funding: NA