Anti-metabotropic glutamate receptor 5 encephalitis: Five case reports and literature review
Sixian Chen1, Haitao Ren1, Fuhong Lin2
1Department of Neurology, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.
Objective:
To describe the clinical and radiological characteristics of anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis.
Methods:
We reviewed the clinical data of five patients with anti-mGluR5 encephalitis, and performed a literature review.
Results:
The five cases included a 52-year-old man who developed a biphasic course of anti-mGluR5 encephalitis after herpes simplex encephalitis, a 22-year-old woman who showed bilateral basal ganglia lesions on brain magnetic resonance imaging (MRI), and a 36-year-old man with mixed aphasia and generalized tonic-clonic seizures, a 51-year-old man presented with personality changes, hallucinations, delusions, sleeping disorders and a 58-year-old man with short-term memory deficits and absence seizures.. There are 16 reported cases of anti-mGluR5 encephalitis worldwide. Of all 21 patients, with a median onset age of 35 years old, the main neurological symptoms were cognitive impairment (85.7%, 18/21), psychiatric or behavior problems (76.2%, 16/21), seizures (57.1%, 12/21), sleeping disorders (52.4%, 11/21), different degrees of decreased consciousness (42.9%, 9/21), and movement disorders (23.8%, 5/21). Brain MRI was normal in 11 of 21 patients. Lesions of the limbic lobes were presented in 5 patients, while involvement of other extralimbic regions was also reported. Seven of 21 (33.3%) cases were combined with tumors. Elevated white blood cell counts or specific oligoclonal IgG bands in the cerebrospinal fluid were found in 18 of 21 patients, with marked improvements observed after immunotherapy.
Discussion:
Patients with anti-mGluR5 encephalitis typically present with diffuse, rather than purely limbic, encephalitis. Anti-mGluR5 encephalitis can be triggered by herpes simplex encephalitis. The risk of a combined tumor may be reduced in anti-mGluR5 encephalitis patients.
Insights
Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis often presents with diffuse brain inflammation, not just limbic involvement. Early immunotherapy can lead to significant patient improvement.
Area of Science:
- Neuroimmunology
- Neurology
Background:
- Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis is a rare autoimmune neurological disorder.
- Understanding its clinical and radiological features is crucial for timely diagnosis and treatment.
Approach:
- This study reviewed five clinical cases of anti-mGluR5 encephalitis.
- A comprehensive literature review of 16 additional cases was performed to consolidate findings.
Key Points:
- The primary symptoms include cognitive impairment, psychiatric disturbances, seizures, and sleep disorders.
- Brain MRI may be normal or show limbic and extralimbic lesions.
- A significant proportion of patients (33.3%) had associated tumors, and immunotherapy showed positive results.
Conclusions:
- Anti-mGluR5 encephalitis typically manifests as diffuse encephalitis, potentially triggered by herpes simplex encephalitis.
- While tumors can co-occur, immunotherapy offers a promising treatment avenue for these patients.
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