Extra–central nervous system target for assessment and treatment in refractory anti–N-methyl-d-aspartate receptor encephalitis

Anti–N-methyl-d-aspartate–type glutamate receptor autoimmune encephalitis can arise in the setting of ovarian teratoma and often responds to resection. When it occurs in the absence of tumor, failure to respond to treatment may be more likely, and affected patients often require intensive care. To f...

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Bibliographic Details
Published in:Journal of critical care 2017-02, Vol.37, p.234-236
Main Author: Nauen, David W.
Format: Article
Language:English
Subjects:
Anti-N-Methyl-D-Aspartate Receptor Encephalitis - immunology
Anti-N-Methyl-D-Aspartate Receptor Encephalitis - pathology
Anti-N-Methyl-D-Aspartate Receptor Encephalitis - therapy
Anti-NMDAR encephalitis
Autoantibodies - immunology
Autopsy
Biopsy
Blood
Brain - pathology
Encephalitis
Enzymes
Female
Herpes viruses
Hippocampus - pathology
Humans
Immunoglobulins
Immunologic Factors - therapeutic use
Infections
Lymphatic system
Lymphocytes
Microglia - immunology
Microglia - pathology
Mortality
Nervous system
Neurointensive care
NMR
Nuclear magnetic resonance
Patients
Plasmapheresis
Polymerase chain reaction
Refractory encephalitis
Rituximab - therapeutic use
T-Lymphocytes - immunology
T-Lymphocytes - pathology
Temporal Lobe - pathology
Young Adult
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Summary:Anti–N-methyl-d-aspartate–type glutamate receptor autoimmune encephalitis can arise in the setting of ovarian teratoma and often responds to resection. When it occurs in the absence of tumor, failure to respond to treatment may be more likely, and affected patients often require intensive care. To further understand the mechanisms and potential management, we present findings from an autopsy conducted on a young woman who died of refractory autoimmune encephalitis of this type. Rituximab was administered 70 days before death, and both 37 and 14 days before death, CD19+ lymphocytes were only 0.1% of blood cells. Ten sessions of plasmapheresis were performed after rituximab treatment. Nonetheless, the autoantibodies were present in serum 4 days before death, demonstrating ongoing antibody production. The hippocampus and medial temporal lobe demonstrated inflammation with T cell and prominent microglial involvement, but no plasma cells or plasmablasts were found there, or anywhere in the brain, despite an extensive search. Examination of lymph node tissue identified many plasma cells along sinusoids. These findings demonstrate that the antibody-producing cells are long-lived and can reside in lymphoid tissue. Awareness of continuing antibody production, the extra–central nervous system site, the indication for cytotoxic therapy, and the potential for biopsy assessment may lead to more effective treatment.
ISSN:0883-9441
1557-8615
DOI:10.1016/j.jcrc.2016.09.016