Hypoglutamatergic state is associated with reduced cerebral glucose metabolism in anti-NMDA receptor encephalitis: a case report.

Abstract

BACKGROUND:

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis was first described in 2005 in association with ovarian teratoma. The diagnostic workup of this common autoimmune encephalitis includes cerebrospinal fluid analysis, electroencephalography, magnetic resonance imaging (MRI), and fluorodeoxyglucose positron emission tomography (FDG-PET). In addition to standard diagnostics, we performed metabolic investigations using proton magnet resonance spectroscopy ((1)H-MRS).

CASE PRESENTATION:

We describe the case of a non-limbic anti-NMDAR encephalitis with a long course of disease (21 months). Laboratory diagnostics showed antibodies against NMDAR; an MRI revealed unspecific findings. (1)H-MRS indicated a hypoglutamatergic state in the left prefrontal cortex associated with a left hemispherical hypometabolism on FDG-PET. Despite the long course of disease, immunosuppressive therapy with methylprednisolone and azathioprine led to almost complete remission of clinical symptoms. Clinical improvement developed in parallel with remarkable normalization of cerebral glucose metabolism on FDG-PET.

CONCLUSION:

This case of long-lasting extra-limbic anti-NMDAR encephalitis is of high clinical relevance. First, it illustrates that a very good outcome is possible even if adequate therapy is started only 21 months after the onset of severe symptoms. Second, it provides valuable insights into the pathophysiology of such anti-NMDAR encephalitis; these insights prove that anti-NMDAR encephalitis is linked not only to hyperglutamatergic signals but also to hypoglutamatergic states. These findings, contradictory at first glance, can be integrated within the model of excitatory/inhibitory imbalance and local area network inhibition.

• Authors: Dominique Endres, Evgeniy Perlov, Oliver Stich, Sebastian Rauer, Simon Maier, Zora Waldkircher, Thomas Lange, Irina Mader, Philipp Tobias Meyer, Ludger Tebartz van Elst
• Journal: BMC psychiatry (BMC Psychiatry) Vol. 15 Pg. 186 (Aug 01 2015) ISSN: 1471-244X [Electronic] England
• PMID: 26231521 (Publication Type: Case Reports, Journal Article)

Chemical References

• Autoantibodies
• Blood Glucose
• Immunosuppressive Agents
• Neuroprotective Agents
• Receptors, N-Methyl-D-Aspartate
• Azathioprine
• Methylprednisolone

Topics

• Adult
• Anti-N-Methyl-D-Aspartate Receptor Encephalitis (diagnosis, drug therapy, metabolism)
• Autoantibodies (metabolism)
• Azathioprine (therapeutic use)
• Blood Glucose (metabolism)
• Drug Therapy, Combination
• Electroencephalography
• Female
• Humans
• Immunosuppressive Agents (therapeutic use)
• Magnetic Resonance Imaging
• Methylprednisolone (therapeutic use)
• Multimodal Imaging (methods)
• Neuroprotective Agents (therapeutic use)
• Positron-Emission Tomography (methods)
• Receptors, N-Methyl-D-Aspartate (immunology)
• Treatment Outcome