A patient with encephalitis associated with NMDA receptor antibodies. Nat Clin Prac 3:291-296

Hospital of the University of Pennsylvania, Philadelphia, PA, USA.
Nature Clinical Practice Neurology (Impact Factor: 7.64). 06/2007; 3(5):291-6. DOI: 10.1038/ncpneuro0493
Source: PubMed


A 34-year-old woman presented with headache, feverish sensation and anxiety, rapidly followed by homicidal ideation, aggressive agitation, seizures, hypoventilation, hyperthermia and prominent autonomic instability requiring intubation and sedation. She developed episodes of hypotension and bradycardia with periods of asystole lasting up to 15 seconds. Upon weaning off sedation, her eyes opened but she was unresponsive to stimuli. There was muscle rigidity, frequent facial grimacing, rhythmic abdominal contractions, kicking motions of the legs, and intermittent dystonic postures of the right arm.
Routine laboratory testing, toxicology screening, studies for autoimmune and infectious etiologies, brain MRI scan, lumbar puncture, electroencephalogram, whole-body CT scan, abdominal ultrasound, paraneoplastic and voltage-gated potassium channel antibody serologies, analysis of N-methyl-D-aspartate receptor antibodies.
Paraneoplastic encephalitis associated with immature teratoma of the ovary and N-methyl-D-aspartate receptor antibodies.
Intensive care, mechanical ventilation, antiepileptics, laparotomy and left salpingo-oophorectomy, corticosteroids, plasma exchange, intravenous immunoglobulin, cyclophosphamide, physical therapy, and chemotherapy.

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Available from: Erdem Tüzün, Jul 11, 2014
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    • "A recently reported case series attempted to differentiate male ANRE from female ANRE and suggested that male patients, similar to our case, present with early seizures, whereas behavioral changes are more common as an early manifestation in female patients [12]. Seizures are a common feature of ANRE [2] [13] [14] and occur in more than two-thirds of patients (76/100) in a recently published large series [1]. A few refractory cases of SE as a result of ANRE have been reported; however, their details were not specified. "
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    ABSTRACT: Anti-NMDA receptor encephalitis (ANRE) has been previously reported as a rare cause of nonconvulsive status epilepticus (NCSE). Vagus nerve stimulation (VNS) is generally considered as a palliative treatment for patients with drug-resistant partial-onset epilepsy. Here, we report a case of refractory NCSE that was terminated after vagus nerve stimulator implantation. To our knowledge, similar cases have not been reported previously.
    Full-text · Article · Dec 2015 · Epilepsy and Behavior Case Reports
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    • "Another feature of anti-NMDAR encephalitis is that it predominantly affects women of reproductive age (Kamei et al. 2009), and is often accompanied by ovarian teratoma (Dalmau et al. 2007, 2008). Mature-and immature-appearing neurons in ovarian teratoma ectopically express NMDARs (Sansing et al. 2007; Seki et al. 2008; Tüzün et al. 2009); this is thought to contribute to the production of anti-NMDAR antibodies. Therefore, anti- NMDAR encephalitis has been considered to be a type of paraneoplastic encephalitis (Vitaliani et al. 2005; Dalmau et al. 2007). "
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    ABSTRACT: Autoimmune synaptic encephalitis is characterized by the presence of autoantibodies against synaptic constituent receptors and manifests as neurological and psychiatric disorders. Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is such an autoimmune disorder that predominantly affects young women. It is associated with antibodies against the extracellular region of the NR1 subunit of postsynaptic NMDAR. Each NMDAR functions as a heterotetrameric complex that is composed of four subunits, including NR1 and NR2A, NR2B, or NR2C. Importantly, ovarian teratoma is a typical complication of anti-NMDAR encephalitis in female patients and may contain antigenic neural tissue; however, antigenic sites remain unknown in female patients without ovarian teratoma. The purpose of this study was to investigate the expression of NMDARs in the ovum. We detected NR1 and NR2B immunoreactivity in protein fractions extracted from the bovine ovary and ova by SDS-polyacrylamide gel electrophoresis and immunoblotting analysis. Immunoprecipitates digested with trypsin were analyzed by reverse phase liquid chromatography coupled to tandem mass spectrometry. We obtained the following five peptides: SPFGRFK and KNLQDR, which are consistent with partial sequences of human NR1, and GVEDALVSLK, QPTVAGAPK, and NEVMSSK, which correspond to those of NR2A, NR2B and NR2C, respectively. Immunocytochemical analysis revealed that the bovine ovum was stained with the immunoglobulin G purified from the serum of a patient with anti-NMDAR encephalitis. Taken together, we propose that the normal ovum expresses NMDARs that have strong affinity for the disease-specific IgG. The presence of NMDARs in ova may help explain why young females without ovarian teratomas are also affected by anti-NMDAR encephalitis.
    Preview · Article · Mar 2015 · The Tohoku Journal of Experimental Medicine
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    • "LGI1 antikoru saptanan hastalarda nöbet gelişiminin, CASPR-2 pozitif olanlarla karşılaştırıldığında daha fazla olduğu görülmüştür.[36]Fasyobrakiyal distonik nöbetlerin görüldüğü hastaların %89'unda LGI1 antikoru saptanmıştır.373839Bu grup hastalar için paantikor saptanan hastaların %75'i immün tedaviye yanıt vermiş ve bunların çoğunda nöronal yüzey antijenlerine karşı gelişen antikorlar saptanmıştır.[25]Antikorlarla "

    Preview · Article · Jan 2015
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