Encephalitis associated with anti-mGluR5 antibodies
Denison Alves Pedrosa1, João Henrique Fregadolli Ferreira1, Rene Gleizer1
1Hospital Israelita Albert Einstein, São Paulo, Brazil.
Abstract:
A 30-year-old woman had 5 days of visual hallucinations, nystagmus, memory impairment and mutism. On examination, she was disorientated with reduced attention span, gaze-evoked nystagmus, paratonia and abnormal frontal reflexes. Cerebrospinal fluid (CSF) showed 80 cells, protein 0.41 g/L and glucose 3.2 mmol/L (plasma glucose 5.0 mmol/L). MR scan of the brain showed involvement of limbic and extra-limbic regions and brainstem. Commercial cell-based assays were negative, but tissue-based assays showed neuropil staining, and cell-based assays for anti-metabotropic glutamate receptor 5 (mGluR5) antibodies were positive in serum and CSF. Six months later, she was diagnosed with Hodgkin's lymphoma. This case emphasises the broader clinical spectrum of anti-mGluR5 encephalitis, challenging its initial characterisation as Ophelia syndrome. It underscores the significance of interpreting commercial cell-based assays and advocates for tissue-based assay testing followed by cell-based assay testing in serum and CSF for diagnosing rare autoimmune encephalitis.
Insights
This case highlights anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis, presenting beyond Ophelia syndrome. Early diagnosis requires advanced antibody testing, not just commercial assays, for rare autoimmune encephalitis.
Area of Science:
- Neurology
- Immunology
- Oncology
Background:
- Autoimmune encephalitis presents with diverse neurological symptoms.
- Anti-metabotropic glutamate receptor 5 (mGluR5) encephalitis is a rare neurological disorder.
- Ophelia syndrome is a previously described presentation of anti-mGluR5 encephalitis.
Purpose of the Study:
- To describe a case of anti-mGluR5 encephalitis with an atypical presentation.
- To emphasize the diagnostic challenges and the importance of specific antibody testing.
- To highlight the association between autoimmune encephalitis and underlying malignancies.
Main Methods:
- Clinical case presentation of a 30-year-old woman with neurological symptoms.
- Cerebrospinal fluid (CSF) and serum analysis, including cell counts, protein, and glucose levels.
- Magnetic Resonance (MR) imaging of the brain.
- Immunological assays: commercial cell-based assays (CBAs), tissue-based assays, and specific CBAs for anti-mGluR5 antibodies.
Main Results:
- The patient exhibited visual hallucinations, nystagmus, memory impairment, and mutism.
- CSF analysis revealed pleocytosis and altered glucose levels.
- Brain MR imaging showed limbic, extra-limbic, and brainstem involvement.
- Tissue-based assays showed neuropil staining, and serum and CSF CBAs for anti-mGluR5 antibodies were positive.
- The patient was later diagnosed with Hodgkin's lymphoma.
Conclusions:
- Anti-mGluR5 encephalitis has a broader clinical spectrum than previously characterized.
- Commercial cell-based assays may be insufficient for diagnosing rare autoimmune encephalitis.
- Tissue-based assays followed by serum and CSF antibody testing are crucial for accurate diagnosis.
- Autoimmune encephalitis can be paraneoplastic, associated with underlying cancers like Hodgkin's lymphoma.
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