Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a form of autoimmune encephalopathy that presents with a wide variety of symptoms, including neuropsychiatric manifestations. The authors' aim for this study was to analyze the results of paraclinical studies of patients with a diagnosis of anti-NMDAR encephalitis and the association between symptom onset and diagnosis, and start of immunotherapy. Retrospective data of 29 patients with anti-NMDAR encephalitis were gathered and analyzed. Abnormal EEG was found in 27 patients (93.1%), whereas MRI was abnormal in 19 patients (65.5%). In contrast, an inflammatory pattern on CSF analysis was found in only 13 patients (44.8%). The absence of pleocytosis or increased proteins in the CSF was associated with a longer time from symptom onset to diagnosis and treatment (p = 0.003). The authors conclude that noninflammatory CSF may delay the correct diagnosis and start of immunotherapy in anti-NMDAR encephalitis. In the presence of suggestive clinical features, extensive studies including EEG are recommended.
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http://dx.doi.org/10.1590/0004-282X20170179 | DOI Listing |
Cureus
December 2024
Neurology, SRM Medical College Hospital and Research Centre, SRM Institute of Science and Technology, Chengalpattu, IND.
Introduction: This study discusses the various clinical profiles, investigatory findings, treatment responses, and prognosticating factors in seven cases of autoimmune encephalitis (AE).
Methods: The clinical records of seven AE patients admitted to the Neurology Department, SRM Medical College Hospital and Research Centre, Chennai, from July 2022 to December 2023 were retrospectively analyzed.
Results: The patients' ages ranged from 18 to 35, and all experienced seizures.
Int J Mol Sci
January 2025
Department of Pathology, Faculty of Health Care and Social Work, Trnava University and University Hospital, 917 02 Trnava, Slovakia.
The autoantibodies against the NR1 subunit are well known in the pathomechanism of NMDAR encephalitis. The dysfunction of the NR2 subunit could be a critical factor in this neurological disorder due to its important role in the postsynaptic pathways that direct synaptic plasticity. We report a case of paraneoplastic anti-NMDAR encephalitis presented alongside very severe illness.
View Article and Find Full Text PDFBMC Neurol
January 2025
Department of Neurology, Liuzhou People's Hospital affiliated to Guangxi Medical University, No.8 Rd.wenchang Liuzhou, Liuzhou, 545000, Guangxi Province, China.
Background: Anti-NMDA receptor encephalitis is an autoimmune, antibody-mediated inflammatory disease of the brain characterized by the presence of IgG antibodies targeting the excitatory N-methyl-D-aspartate receptor (NMDAR). Previous research has established that the neonatal Fc receptor (FcRn) regulates the transport and circulation of immunoglobulins (IgG). Efgartigimod, an FcRn antagonist, has been shown to enhance patient outcomes by promoting IgG clearance, and it has exhibited substantial clinical efficacy and tolerability in the treatment of myasthenia gravis.
View Article and Find Full Text PDFBackground: Anti-N-methyl-D-aspartic receptor encephalitis (Anti-NMDAR encephalitis) is the most prevalent form of autoimmune encephalitis in pediatric patients. Autonomic dysfunction is a frequent symptom of Anti-NMDAR encephalitis, yet it often goes unnoticed by pediatricians. Studies have indicated that pediatric patients with autonomic dysfunction exhibit a poorer prognosis compared to those without.
View Article and Find Full Text PDFNeurol Sci
January 2025
Department of Neurology, Tongji Medical College, Union Hospital, Huazhong University of Science and Technology, Wuhan, China.
The Fc receptor (FcRn) inhibitors can ameliorate autoimmune conditions such as myasthenia gravis through a rapid and specific clearance of serum IgG levels, and they also have potential for future use in a wider variety of antibody-mediated autoimmune diseases. Some patients with therapy-refractory autoimmune encephalitis (AE) continue to be unresponsive to initial and secondary treatment regimens. A 32-year-old male presented with predominant psychiatric symptoms and seizures, along with imaging evidence indicating multifocal cerebral cortical involvement.
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