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FLAMES with Elevated Myelin Basic Protein Followed by Myelitis
Hiroyuki Hokama1, Yuki Sakamoto1, Toshiyuki Hayashi1
1Department of Neurology, Graduate School of Medicine, Nippon Medical School, Japan.
Abstract:
The pathophysiology of unilateral cortical fluid-attenuated inversion recovery (FLAIR)-hyperintense lesions in anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis with seizures (FLAMES) is unclear. A 26-year-old man was referred because of a seizure. FLAIR showed an increased signal intensity and swelling of the right frontal cortex. His symptoms and imaging abnormalities were improved after intravenous methylprednisolone therapy. MOG antibody was detected both in serum and cerebrospinal fluid (CSF). Therefore, the patient was diagnosed with FLAMES. Myelin basic protein (MBP) was elevated in CSF. The high MBP value in the CSF in the present case suggested that demyelination as well as inflammation can occur in some FLAMES patients.
Insights
This study investigates anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis with seizures (FLAMES), revealing potential demyelination alongside inflammation. Elevated myelin basic protein in CSF suggests this dual pathology in FLAMES patients.
Area of Science:
- Neuroimmunology
- Neurology
- Neuroinflammation
Background:
- The underlying mechanisms of unilateral cortical lesions in anti-myelin oligodendrocyte glycoprotein (MOG)-associated encephalitis with seizures (FLAMES) remain poorly understood.
- FLAMES is an inflammatory neurological condition characterized by seizures and specific brain imaging findings.
Observation:
- A 26-year-old male presented with seizures and unilateral cortical FLAIR hyperintense lesions.
- Cerebrospinal fluid (CSF) analysis revealed elevated myelin basic protein (MBP) and detected MOG antibodies.
- The patient showed significant improvement following intravenous methylprednisolone treatment.
Findings:
- The presence of MOG antibodies in serum and CSF confirmed the diagnosis of FLAMES.
- Elevated MBP levels in the CSF suggest that demyelination is a component of the inflammatory process in this FLAMES case.
- The findings indicate that both inflammation and demyelination may contribute to the pathophysiology of FLAMES.
Implications:
- This case highlights the potential for demyelination in FLAMES, expanding our understanding of the disease spectrum.
- The results suggest that therapeutic strategies targeting both inflammation and demyelination might be beneficial for FLAMES patients.
- Further research is warranted to elucidate the precise role of demyelination in FLAMES and its impact on patient outcomes.
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