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Hypoglutamatergic state is associated with reduced cerebral glucose metabolism in anti-NMDA receptor encephalitis: a case report.
BMC Psychiatry. 2015 Aug 01; 15:186.BP

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+Show Affiliations

Section of Experimental Neuropsychiatry, Department for Psychiatry& Psychotherapy, University Medical Center Freiburg, Hauptstr. 5, 79104, Freiburg, Germany. dominique.endres@uniklinik-freiburg.de. Freiburg Brain Imaging, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. dominique.endres@uniklinik-freiburg.de.Section of Experimental Neuropsychiatry, Department for Psychiatry& Psychotherapy, University Medical Center Freiburg, Hauptstr. 5, 79104, Freiburg, Germany. evgeniy.perlov@uniklinik-freiburg.de. Freiburg Brain Imaging, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. evgeniy.perlov@uniklinik-freiburg.de.Department of Neurology, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. oliver.stich@uniklinik-freiburg.de.Department of Neurology, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. sebastian.rauer@uniklinik-freiburg.de.Section of Experimental Neuropsychiatry, Department for Psychiatry& Psychotherapy, University Medical Center Freiburg, Hauptstr. 5, 79104, Freiburg, Germany. simon.maier@uniklinik-freiburg.de. Freiburg Brain Imaging, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. simon.maier@uniklinik-freiburg.de.Section of Experimental Neuropsychiatry, Department for Psychiatry& Psychotherapy, University Medical Center Freiburg, Hauptstr. 5, 79104, Freiburg, Germany. z.waldkircher@park-klinikum.de.Department of Radiology, Medical Physics, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. thomas.lange@uniklinik-freiburg.de.Department of Neuroradiology, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. irina.mader@uniklinik-freiburg.de.Department of Nuclear Medicine, University Medical Center Freiburg, Hugstetter Str. 64, 79106, Freiburg, Germany. philipp.meyer@uniklinik-freiburg.de.Section of Experimental Neuropsychiatry, Department for Psychiatry& Psychotherapy, University Medical Center Freiburg, Hauptstr. 5, 79104, Freiburg, Germany. tebartzvanelst@uniklinik-freiburg.de. Freiburg Brain Imaging, University Medical Center Freiburg, Breisacher Str. 64, 79106, Freiburg, Germany. tebartzvanelst@uniklinik-freiburg.de.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

26231521

Citation

Endres, Dominique, et al. "Hypoglutamatergic State Is Associated With Reduced Cerebral Glucose Metabolism in anti-NMDA Receptor Encephalitis: a Case Report." BMC Psychiatry, vol. 15, 2015, p. 186.
Endres D, Perlov E, Stich O, et al. Hypoglutamatergic state is associated with reduced cerebral glucose metabolism in anti-NMDA receptor encephalitis: a case report. BMC Psychiatry. 2015;15:186.
Endres, D., Perlov, E., Stich, O., Rauer, S., Maier, S., Waldkircher, Z., Lange, T., Mader, I., Meyer, P. T., & van Elst, L. T. (2015). Hypoglutamatergic state is associated with reduced cerebral glucose metabolism in anti-NMDA receptor encephalitis: a case report. BMC Psychiatry, 15, 186. https://doi.org/10.1186/s12888-015-0552-4
Endres D, et al. Hypoglutamatergic State Is Associated With Reduced Cerebral Glucose Metabolism in anti-NMDA Receptor Encephalitis: a Case Report. BMC Psychiatry. 2015 Aug 1;15:186. PubMed PMID: 26231521.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Hypoglutamatergic state is associated with reduced cerebral glucose metabolism in anti-NMDA receptor encephalitis: a case report. AU - Endres,Dominique, AU - Perlov,Evgeniy, AU - Stich,Oliver, AU - Rauer,Sebastian, AU - Maier,Simon, AU - Waldkircher,Zora, AU - Lange,Thomas, AU - Mader,Irina, AU - Meyer,Philipp Tobias, AU - van Elst,Ludger Tebartz, Y1 - 2015/08/01/ PY - 2015/02/02/received PY - 2015/07/06/accepted PY - 2015/8/2/entrez PY - 2015/8/2/pubmed PY - 2016/1/5/medline SP - 186 EP - 186 JF - BMC psychiatry JO - BMC Psychiatry VL - 15 N2 - 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. SN - 1471-244X UR - https://www.unboundmedicine.com/medline/citation/26231521/Hypoglutamatergic_state_is_associated_with_reduced_cerebral_glucose_metabolism_in_anti_NMDA_receptor_encephalitis:_a_case_report_ L2 - https://bmcpsychiatry.biomedcentral.com/articles/10.1186/s12888-015-0552-4 DB - PRIME DP - Unbound Medicine ER -