Clinical experience and laboratory investigations in patients with anti-NMDAR encephalitis

By J. Dalmau, E. Lancaster, E. Martínez-Hernandez, M. Rosenfeld, R. Balice-Gordon. Lancet Neurology, 2011

2,244 citations recorded by the source corpus.

Since its discovery in 2007, the encephalitis associated with antibodies against the N-methyl-D-aspartate receptor (NMDAR) has entered the mainstream of neurology and other disciplines. Most patients with anti-NMDAR encephalitis develop a multistage illness that progresses from psychosis, memory deficits, seizures, and language disintegration into a state of unresponsiveness with catatonic features often associated with abnormal movements, and autonomic and breathing instability. The disorder predominantly affects children and young adults, occurs with or without tumour association, and responds to treatment but can relapse. The presence of a tumour (usually an ovarian teratoma) is dependent on age, sex, and ethnicity, being more frequent in women older than 18 years, and slightly more predominant in black women than it is in white women. Patients treated with tumour resection and immunotherapy (corticosteroids, intravenous immunoglobulin, or plasma exchange) respond faster to treatment and less frequently need second-line immunotherapy (cyclophosphamide or rituximab, or both) than do patients without a tumour who receive similar initial immunotherapy. More than 75% of all patients have substantial recovery that occurs in inverse order of symptom development and is associated with a decline of antibody titres. Patients’ antibodies cause a titre-dependent, reversible decrease of synaptic NMDAR by a mechanism of crosslinking and internalisation. On the basis of models of pharmacological or genetic disruption of NMDAR, these antibody effects reveal a probable pathogenic relation between the depletion of receptors and the clinical features of anti-NMDAR encephalitis.

Subfields: Genetics, Neurology

View Paper: https://www.semanticscholar.org/paper/6187338e58a869690b35fa848f1713c6dc6d87ae

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Citations in the corpus

Immunopathology of autoantibody-associated encephalitides: clues for pathogenesis. (2012, background): “…and also in anti-NMDAR encephalitis in which removal of an underlying malignancy (usually ovarian teratoma) can be associated with excellent recovery; clinical outcomes in the patients without detectable tumours are less complete and some relapse (Irani et al., 2010a; Dalmau et al., 2011).”
The frequency of autoimmune N-methyl-D-aspartate receptor encephalitis surpasses that of individual viral etiologies in young individuals enrolled in the California Encephalitis Project. (2012, background): “initially thought to affect primarily young adult females, often with associated teratomas, it was later described in men and children, frequently without any identifiable tumor [2].”
Case definitions, diagnostic algorithms, and priorities in encephalitis: consensus statement of the international encephalitis consortium. (2013, background): “…is characterized by brain in ﬂ ammation as a consequence of direct infection of the brain parenchyma, a post-infectious process such as acute disseminated encephalomy-elitis (ADEM) [6, 15], or a noninfectious condition such as anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis [16, 17].”
Sugar for the brain: the role of glucose in physiological and pathological brain function (2013, background): “The symptoms start with fever, psychosis, and seizures, and progress to abnormal movements, respiratory failure, dysautonomia, and coma [102].”