The issue of persistence of NMDAR antibodies after encephalitis is not fully elucidated and their relationship with demyelinating disorders has been suggested. A female patient showed at the age of 18 an acute neurological disorder (with psychiatric symptoms, focal seizures, orofacial dyskinesias and hypoventilation requiring ventilatory support) clinically mimicking anti-NMDAR encephalitis. At that time specific laboratory tests were not available, CSF revealed oligoclonal bands and MRI was negative. The patient had full recovery after first line immunotherapy (i.v. steroids and immunoglobulins). Fifteen years later, at the age of 33, she was hospitalized with subacute right hemiparesis and MRI disclosed multiple T2 hyperintensities in the white matter, one of them in the left midbrain showing contrast enhancement. Serum and CSF NMDAR antibodies were positive while MOG and AQP4 antibodies were negative. Intravenous methylprednisolone led to complete recovery. This case report provides evidence of a long-term persistence of NMDAR antibodies even 15 years after the encephalitis and raises the suspicion of a possible causal relationship between NMDAR antibodies and demyelinating disorders in the form of multiple sclerosis.
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http://dx.doi.org/10.1007/s10072-020-04729-3 | DOI Listing |
J Clin Neurophysiol
October 2024
Clinical Neurophysiology Department, National Institute of Neurology and Neurosurgery, Mexico City, Mexico.
Purpose: Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune reaction involving Immunoglobulin G antibodies against GluN1 subunit of NMDAR. Absence of biomarkers for early diagnosis and prognosis poses a challenge. Several small case-control studies have emphasized the prospect of quantitative EEG measurements.
View Article and Find Full Text PDFBrain
December 2024
Neuroimmunology Program, Fundació Clínic per la Recerca Biomèdica - Institut d'Investigacions Biomèdiques August Pi i Sunyer (FCRB-IDIBAPS), Barcelona 08036, Spain.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a disorder mediated by autoantibodies against the GluN1 subunit of NMDAR. It occurs with severe neuropsychiatric symptoms that often improve with immunotherapy. Clinical studies and animal models based on patients' antibody transfer or NMDAR immunization suggest that the autoantibodies play a major pathogenic role.
View Article and Find Full Text PDFCureus
November 2024
Neurology, National Institute of Neurology and Neurosurgery "Dr. Manuel Velasco Suárez", Mexico City, MEX.
Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis stands as the most prevalent form of autoimmune encephalitis, primarily affecting young patients and exhibiting a higher incidence among females. Patients frequently present with psychiatric symptoms or cognitive impairments such as speech disturbances, decreased level of consciousness, autonomic dysfunction, as well as seizures, dyskinesias, and catatonia due to overactivation of extrasynaptic NMDA receptors. To date, there is no gold standard for the diagnosis of catatonia; however, a few rating scales exist to measure this phenomenon, with the Bush Francis Catatonia Rating Scale being the most commonly used due to its validity, reliability, and ease of application.
View Article and Find Full Text PDFEur J Paediatr Neurol
December 2024
Department of Brain & Neurosciences, Tokyo Metropolitan Institute of Medical Science, Tokyo, Japan. Electronic address:
Objective: Early diagnosis and treatment of anti-N-methyl-D-aspartate receptor encephalitis (NMDARE) are crucial for a favorable prognosis. Detecting the causative autoantibodies can be challenging. Probable diagnostic criteria are useful in adults less so in children.
View Article and Find Full Text PDFAnn Clin Transl Neurol
December 2024
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
Objective: We describe neurologic phenotype, clinical associations, and outcomes in autoimmune brainstem encephalitis.
Methods: Medical records of neural-IgG positive autoimmune brainstem encephalitis patients diagnosed at Mayo Clinic (January 1, 2006-December 31, 2022) were reviewed.
Results: Ninety-eight patients (57 male) were included.
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