Letter to the EditorA case of anti-NMDA receptor encephalitis in a woman with a NMDA-R+ small cell lung carcinoma (SCLC)
Introduction
Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder characterized by IgG autoantibodies directed against the NR1 subunit of the NMDA glutamate receptor [1]. The disease commonly occurs in young females and in this range of sex and age is associated in about 50% of cases with ovarian teratoma [1], [2], [3]. Cases have also been reported in older patients, predominantly men, but cancer is less frequent in this age range [4]. Other tumors than teratoma are rare. They represent less than 5% of cases [1] and can develop from a wide range of organ [1], [3]. We report here a case of anti-NMDAR encephalitis associated with small cell lung cancer (SCLC).
Section snippets
Case history
A 62 years old, heavy smoker (30 packs/year), woman experienced mood and behavioral changes four weeks prior to hospitalization in the intensive care unit. She was diagnosed with a severe depression and received antidepressant, benzodiazepines and neuroleptic treatments but her condition rapidly deteriorated with catatonic state, mutism, oppositional attitude, followed by complex partial seizures, orofacial dyskinesia, dystonic movements in the left side of the body and finally severe
Small cell lung cancer cells immunoreactivity with the patient serum
Detection of autoantibodies against the NR1 subunit of the NMDA glutamate receptor was performed in the serum or CSF by indirect immunofluorescence with NMDAR HEK transfected cells (Euroimmun, Germany). IgG from the patient and from a control normal subject diluted 1:10 in PBS-Tween 0.1% and revealed with FITC labelled anti-human IgG were used to detect NMDAR expression in the patient's tumor and in four SCLC from patients without neurological syndrome. Tumor cells were taken from lymph node
Discussion
The case reported here had the typical clinical and chronological pattern of anti-NMDAR AB encephalitis marked by initial psychiatric symptoms followed within a few weeks by loss of consciousness, seizures, dysautonomia, abnormal movements and central hypoventilation [3]. Extreme delta brush on EEG was also typical of the disorder [5] and anti-NMDAR antibodies were detected both in the serum and the CSF. However, this case displays several unusual features. First the encephalitis affected a
Conflict of interest
No conflict of interest.
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2020, Clinical ImmunologyCitation Excerpt :NMDARs are firstly and primarily found in neurons and play an important role in the development of neurons and synaptic plasticity during brain development [2,3]. They are also found in neuralglial cells [4], brain endothelial cells [5], peripheral non-neural tissues [6,7], and carcinomas [8–13], especially in teratomas rich in NMDAR-bearing neural tissues [14–16]. Anti-NMDAR encephalitis is an autoimmune encephalitis mediated by anti-NMDAR antibodies that present with a subacute onset of individually variant symptoms such as psychiatric abnormalities, seizures, and involuntary movement followed by autonomic instability and disturbed consciousness.
Neuroendocrine Neoplasms of the Lung
2018, Practical Pulmonary Pathology: A Diagnostic Approach A Volume in the Pattern Recognition SeriesAnti-NMDA Receptor Encephalitis: Clinical Features and Basic Mechanisms
2018, Advances in PharmacologyCitation Excerpt :Interestingly, isolated seizures as a presentation are more frequent in men than women for reasons that are unclear. Similarly, a large number of novel tumors are associated with anti-NMDAR, including hepatic neuroendocrine tumors, uterine neuroendocrine tumors, small cell lung cancer, and carcinoma of unknown origin (Afanasiev et al., 2016; Hara et al., 2011; Jeraiby, Depincé-Berger, Bossy, Antoine, & Paul, 2016; Wu, He, Zhang, Shi, & Zhang, 2016). The pathology of anti-NMDARE is consistent with a paraneoplastic limbic encephalitis.