In the majority of cases, anti-NMDA (N-methyl-D-aspartate) receptor encephalitis is a severe, but treatable disorder, therefore early diagnosis and adequate therapy are very important. It should be suspected in children and young women, who develop acute psychiatric symptoms and seizures. During the course of the disease severe encephalopathy, agitation, hallucinations, orofacial dyskinesias, prolonged cognitive disturbance, autonomic symptoms can be observed and akinetic mutism develops. EEG shows diffuse slowing. Brain MRI is normal or unspecific. Elevated protein, pleiocytosis and oligoclonal bands can be present in the CSF Detection of NMDA-receptor antibodies in sera or CSF confirms diagnosis. We present the case of a 15-year-old girl, who fully recovered within two months after steroid treatment and repeated plasma exchange. Ovarian teratoma has not been detected.
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Pediatr Int
January 2025
Department of Pediatrics, Fujita Health University School of Medicine, Toyoake, Japan.
Eur J Neurosci
January 2025
Department of Neurology, the Second Xiangya Hospital, Central South University, Changsha, Hunan, China.
To summarise the clinical characteristics, radiological features, treatments and prognosis of patients with myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) overlapped with NMDA receptor (NMDAR) encephalitis. We retrospectively analysed patients who exhibited dual positivity for MOG antibodies and NMDAR antibodies in serum/CSF from Jan 2018 to Jun 2023. Ten patients with MOGAD and NMDAR encephalitis were enrolled.
View Article and Find Full Text PDFClin Transl Med
January 2025
Department of Neurology, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, China.
Background: Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is a prevalent type of autoimmune encephalitis caused by antibodies targeting the NMDAR's GluN1 subunit. While significant progress has been made in elucidating the pathophysiology of autoimmune diseases, the immunological mechanisms underlying anti-NMDARE remain elusive. This study aimed to characterize immune cell interactions and dysregulation in anti-NMDARE by leveraging single-cell multi-omics sequencing technologies.
View Article and Find Full Text PDFJ Neurol
January 2025
Department of Neurology, Mayo Clinic, Rochester, MN, 55905, USA.
Background: Seizures, including status epilepticus (SE), are common in anti-NMDA receptor encephalitis (NMDARE). We aimed to describe clinical and electrographic features of patients with seizures with NMDARE, determine factors associated with SE, and describe long-term seizure outcomes.
Methods: We retrospectively identified patients with seizures in the setting of NMDARE treated at inpatient Mayo Clinic sites during the acute phase of encephalitis between October 2008 and March 2023.
Pharmaceuticals (Basel)
December 2024
School of Pharmacy, University of Reading, Whiteknights, Reading RG6 6AJ, UK.
Recent studies, typically using patient cerebrospinal fluid (CSF), have suggested that different autoantibodies (Aabs) acting on their respective receptors, may underlie neuropsychiatric disorders. The GluN1 (NR1) subunit of the N-methyl-D-aspartate receptor (NMDAR) has been identified as a target of anti-NMDAR Aabs in a number of central nervous system (CNS) diseases, including encephalitis and autoimmune epilepsy. However, the role or the nature of Aabs responsible for effects on neuronal excitability and synaptic plasticity is yet to be established fully.
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