Encephalitis associated with anti-mGluR5 antibodies

Pract Neurol. 2024 Jul 16;24(4):306-309. doi: 10.1136/pn-2024-004089.

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.

Keywords: HALLUCINATIONS; IMAGE ANALYSIS; NEUROIMMUNOLOGY; PARANEOPLASTIC SYNDROME.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Autoantibodies* / blood
  • Autoantibodies* / cerebrospinal fluid
  • Autoantibodies* / immunology
  • Encephalitis* / blood
  • Encephalitis* / diagnosis
  • Encephalitis* / immunology
  • Female
  • Hodgkin Disease / complications
  • Hodgkin Disease / immunology
  • Humans
  • Receptor, Metabotropic Glutamate 5* / immunology

Substances

  • Receptor, Metabotropic Glutamate 5
  • Autoantibodies
  • GRM5 protein, human