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Clinical features and brain MRI volumetric changes in anti-mGluR5 encephalitis
Yueqian Sun1,2, James X Tao3, Xiong Han4
1Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, Beijing, China.
Background:
Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis is a rare and under-recognized autoimmune encephalitis. This study is conducted to characterize its clinical and neuroimaging features.
Methods:
Twenty-nine patients with anti-mGluR5 encephalitis (15 new cases identified in this study and 14 previously reported cases) were included in this study and their clinical features were characterized. Brain MRI volumetric analysis using FreeSurfer software was performed in 9 new patients and compared with 25 healthy controls at both early (≤6 months of onset) and chronic (>1 year of onset) disease stages.
Results:
The common clinical manifestations of anti-mGluR5 encephalitis included cognitive deficits (n = 21, 72.4%), behavioral and mood disturbances (n = 20, 69%), seizures (n = 16, 55.2%), and sleep disorder (n = 13, 44.8%). Tumors were observed in 7 patients. Brain MRI T2/FLAIR signal hyperintensities were observed predominantly in mesiotemporal and subcortical regions in 75.9% patients. MRI volumetric analysis demonstrated significant amygdala enlargement in both early and chronic disease stages compared to healthy controls (P < 0.001). Twenty-six patients had complete or partial recovery, one remained stable, one died and one was lost to follow-up.
Conclusion:
Our findings demonstrated that cognitive impairment, behavioral disturbance, seizures, and sleep disorder are the prominent clinical manifestations of anti-mGluR5 encephalitis. Most patients showed a good prognosis with full recovery, even in the paraneoplastic disease variants. The amygdala enlargement in the early and chronic disease stages is a distinct MRI feature, which exploratively offer a valuable perspective for the study of the disease processes.
Insights
Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis presents with cognitive deficits, behavioral changes, and seizures. Amygdala enlargement on MRI is a key feature, and most patients experience good recovery.
Area of Science:
- Neurology
- Immunology
- Neuroscience
Background:
- Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis is a rare autoimmune neurological disorder.
- It is often under-recognized, necessitating further characterization of its clinical and neuroimaging profiles.
Purpose of the Study:
- To characterize the clinical manifestations of anti-mGluR5 encephalitis.
- To identify distinct neuroimaging features, including volumetric changes, associated with the disease.
Main Methods:
- Retrospective analysis of 29 patients with anti-mGluR5 encephalitis (15 new, 14 previously reported).
- Clinical features were systematically documented.
- Brain MRI volumetric analysis using FreeSurfer was performed on 9 new patients and compared with 25 healthy controls at early and chronic stages.
Main Results:
- Common symptoms include cognitive deficits (72.4%), behavioral/mood disturbances (69%), seizures (55.2%), and sleep disorders (44.8%).
- MRI revealed T2/FLAIR hyperintensities in mesiotemporal and subcortical regions in 75.9% of patients.
- Significant amygdala enlargement was observed in both early and chronic stages compared to controls (P < 0.001).
- Tumors were identified in 7 patients.
Conclusions:
- Cognitive impairment, behavioral disturbances, seizures, and sleep disorders are prominent clinical features.
- Anti-mGluR5 encephalitis generally has a good prognosis, with most patients achieving full recovery, even in paraneoplastic cases.
- Amygdala enlargement represents a distinct MRI finding with potential implications for understanding disease processes.
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