Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma.

Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Research Abstract Details 

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Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Abstract Text:

OBJECTIVE: To report the autoantigens of a new category of treatment-responsive paraneoplastic encephalitis. METHODS: Analysis of clinical features, neuropathological findings, tumors, and serum/cerebrospinal fluid antibodies using rat tissue, neuronal cultures, and HEK293 cells expressing subunits of the N-methyl-D-aspartate receptor (NMDAR). RESULTS: Twelve women (14-44 years) developed prominent psychiatric symptoms, amnesia, seizures, frequent dyskinesias, autonomic dysfunction, and decreased level of consciousness often requiring ventilatory support. All had serum/cerebrospinal fluid antibodies that predominantly immunolabeled the neuropil of hippocampus/forebrain, in particular the cell surface of hippocampal neurons, and reacted with NR2B (and to a lesser extent NR2A) subunits of the NMDAR. NR2B binds glutamate and forms heteromers (NR1/NR2B or NR1/NR2A/NR2B) that are preferentially expressed in the adult hippocampus/forebrain. Expression of functional heteromers (not single subunits) was required for antibody binding. Eleven patients had teratoma of the ovary (six mature) and one a mature teratoma in the mediastinum; five of five tumors examined contained nervous tissue that strongly expressed NR2 subunits and reacted with patients' antibodies. Tumor resection and immunotherapy resulted in improvement or full recovery of eight of nine patients (paralleled by decreased antibody titers); two of three patients without tumor resection died of neurological deterioration. Autopsies showed extensive microgliosis, rare T-cell infiltrates, and neuronal degeneration predominantly involving, but not restricted to, the hippocampus. INTERPRETATION: Antibodies to NR2B- and NR2A-containing heteromers of the NMDAR associate with a severe but treatment-responsive encephalitis. Our findings provide a diagnostic test and suggest a model of autoimmune NMDAR-related encephalitis with broad implications for other immune-mediated disorders of memory, behavior, and cognition.

Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Publishing Authors By Initials

For similar diagnosis: prognosis: treatment outcome research abstracts see: diagnosis: prognosis: treatment outcome research

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Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Journal Published:

PUBLICATION TYPE: Research Support, N.I.H., Extr

Journal: Annals of neurology

VOLUME: 61

Page Numbers: 25-36

Journal Abbreviation: Ann. Neurol.

ISSN: 0364-5134

DAY: 3

MONTH: Jan

YEAR: 2007

Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 7707449

Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Keywords Mesh Terms:

KEYWORDS: Treatment Outcome

MESH TERMS: methods

Chemical & Substance for Abstract: Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma. Information

Substance Name: Receptors, N-Methyl-D-Aspartate

Registry Number: 0

Grant and Affiliation Information for Paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis associated with ovarian teratoma.

AFFILIATION: Department of Neurology, Division of Neuro-oncology, University of Pennsylvania, Philadelphia, PA 19104, USA. josep.dalmau@uphs.upenn.edu

Country: United States

AGENCY: United States NCI

GRANT: R01CA89054

ACRONYM: CA

MEDLINETA: Ann Neurol

REFSOURCE: Ann Neurol. 2007 Jan;61(1):3-4

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