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Myelin Oligodendrocyte Glycoprotein Antibody-associated Cerebral Cortical Encephalitis: Two Cases Highlighting
Rin Yagishita1, Kohei Takikawa1, Riku Toba1
1Department of Neurology, Iwate Prefectural Central Hospital, Japan.
Abstract:
Cerebral cortical encephalitis (CCE) represents a distinct phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease. The most frequent presentation of CCE is acute encephalitis. We describe two cases with diverse clinical courses that posed diagnostic challenges. The cases included a 24-year-old man with a seizure-predominant smoldering course over several months, initially diagnosed with focal epilepsy, and a 27-year-old woman with nonspecific headache and fever without any neurological deficits or seizures. Brain magnetic resonance imaging (MRI) in both cases showed cortical fluid-attenuated inversion recovery hyperintensity with adjacent subcortical hypointensity. CCE may present with diverse clinical courses, and characteristic MRI findings were pivotal for making an accurate diagnosis.
Insights
Cerebral cortical encephalitis (CCE), a form of MOG antibody disease, can present unusually. Characteristic MRI findings are key to diagnosing CCE, even with atypical symptoms like seizures or fever.
Area of Science:
- Neurology
- Immunology
- Radiology
Background:
- Cerebral cortical encephalitis (CCE) is a specific presentation of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease.
- Acute encephalitis is the most common clinical manifestation of CCE.
Purpose of the Study:
- To highlight the diverse clinical presentations of CCE.
- To emphasize the diagnostic utility of MRI findings in CCE.
Main Methods:
- Case report analysis of two patients with CCE.
- Review of clinical courses, diagnostic challenges, and neuroimaging findings.
Main Results:
- One patient presented with a prolonged, seizure-predominant smoldering course mimicking focal epilepsy.
- The second patient had nonspecific symptoms of headache and fever without neurological deficits.
- Both cases exhibited characteristic MRI findings: cortical FLAIR hyperintensity with adjacent subcortical hypointensity.
Conclusions:
- CCE can exhibit varied clinical courses, challenging initial diagnoses.
- Characteristic MRI findings are crucial for accurate and timely diagnosis of CCE.
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