Dataset Information

Teenager with acute psychosis due to non-paraneoplastic anti-N-methyl-d-aspartate receptor encephalitis with a successful recovery: A case report.

ABSTRACT:

Introduction

Anti NMDAR encephalitis is a neuropsychiatric syndromic disease caused by an immunological response. Acute behavioral changes, psychosis, and catatonia are common clinical manifestations, are seizures, amnesia, speech difficulties, dyskinesia, and autonomic dysregulation.

Case presentation

We discuss the case of a 14-year-old girl who had psychotic symptoms and tested positive for anti-NMDAR antibodies.

Discussion

Patients present with psychiatric symptoms such as delusions, hallucinations, agitation, changes in speech mania, disorganized thinking, catatonia, insomnia, and often seizures. Anti-NMDAR encephalitis should be suspected in teenage patient with acute psychotic symptoms and seizure episodes. A multidisciplinary treatment strategy is required.

Conclusion

The delayed treatment can lead to complications and delayed recovery complicating the disease process so multidisciplinary approach of treatment is necessary.

SUBMITTER: Chamlagain R

PROVIDER: S-EPMC9577962 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Teenager with acute psychosis due to non-paraneoplastic anti-N-methyl-d-aspartate receptor encephalitis with a successful recovery: A case report.

Chamlagain Rajan R Shah Sangam S Thapa Sangharsha S Basnet Madan M Kandel Bipin B Paudel Basanta Sharma BS Dhital Roman R Pokhrel Apil A Khanal Pitambar P Paudel Sandip S

Annals of medicine and surgery (2012) 20220928

<h4>Introduction</h4>Anti NMDAR encephalitis is a neuropsychiatric syndromic disease caused by an immunological response. Acute behavioral changes, psychosis, and catatonia are common clinical manifestations, are seizures, amnesia, speech difficulties, dyskinesia, and autonomic dysregulation.<h4>Case presentation</h4>We discuss the case of a 14-year-old girl who had psychotic symptoms and tested positive for anti-NMDAR antibodies.<h4>Discussion</h4>Patients present with psychiatric symptoms such ...[more]

PMID: 36268402

Similar Datasets

Project description:ObjectiveTo report the autoantigens of a new category of treatment-responsive paraneoplastic encephalitis.MethodsAnalysis of clinical features, neuropathological findings, tumors, and serum/cerebrospinal fluid antibodies using rat tissue, neuronal cultures, and HEK293 cells expressing subunits of the N-methyl-D-aspartate receptor (NMDAR).ResultsTwelve women (14-44 years) developed prominent psychiatric symptoms, amnesia, seizures, frequent dyskinesias, autonomic dysfunction, and decreased level of consciousness often requiring ventilatory support. All had serum/cerebrospinal fluid antibodies that predominantly immunolabeled the neuropil of hippocampus/forebrain, in particular the cell surface of hippocampal neurons, and reacted with NR2B (and to a lesser extent NR2A) subunits of the NMDAR. NR2B binds glutamate and forms heteromers (NR1/NR2B or NR1/NR2A/NR2B) that are preferentially expressed in the adult hippocampus/forebrain. Expression of functional heteromers (not single subunits) was required for antibody binding. Eleven patients had teratoma of the ovary (six mature) and one a mature teratoma in the mediastinum; five of five tumors examined contained nervous tissue that strongly expressed NR2 subunits and reacted with patients' antibodies. Tumor resection and immunotherapy resulted in improvement or full recovery of eight of nine patients (paralleled by decreased antibody titers); two of three patients without tumor resection died of neurological deterioration. Autopsies showed extensive microgliosis, rare T-cell infiltrates, and neuronal degeneration predominantly involving, but not restricted to, the hippocampus.InterpretationAntibodies to NR2B- and NR2A-containing heteromers of the NMDAR associate with a severe but treatment-responsive encephalitis. Our findings provide a diagnostic test and suggest a model of autoimmune NMDAR-related encephalitis with broad implications for other immune-mediated disorders of memory, behavior, and cognition.

Project description:Antibodies to the N-methyl-d-aspartate subtype of glutamate receptor have been associated with a newly-described encephalopathy that has been mainly identified in young females with ovarian tumours. However, the full clinical spectrum and treatment responses are not yet clear. We established a sensitive cell-based assay for detection of N-methyl-d-aspartate receptor antibodies in serum or cerebrospinal fluid, and a quantitative fluorescent immunoprecipitation assay for serial studies. Although there was marked intrathecal synthesis of N-methyl-d-aspartate receptor antibodies, the absolute levels of N-methyl-d-aspartate receptor antibodies were higher in serum than in cerebrospinal fluid. N-methyl-d-aspartate receptor antibodies were of the immunoglobulin G1 subclass and were able to activate complement on N-methyl d-aspartate receptor-expressing human embryonic kidney cells. From questionnaires returned on 44 N-methyl-d-aspartate receptor antibody-positive patients, we identified a high proportion without a detected tumour (35/44, 80%: follow-up 3.6-121 months, median 16 months). Among the latter were 15 adult females (43%), 10 adult males (29%) and 10 children (29%), with four in the first decade of life. Overall, there was a high proportion (29%) of non-Caucasians. Good clinical outcomes, as defined by reductions in modified Rankin scores, correlated with decreased N-methyl-d-aspartate receptor antibody levels and were associated with early (<40 days) administration of immunotherapies in non-paraneoplastic patients (P < 0.0001) and earlier tumour removal in paraneoplastic patients (P = 0.02). Ten patients (23%) who were first diagnosed during relapses had no evidence of tumours but had received minimal or no immunotherapy during earlier episodes. Temporal analysis of the onset of the neurological features suggested progression through two main stages. The time of onset of the early features, characterized by neuropsychiatric symptoms and seizures preceded by a median of 10-20 days, the onset of movement disorders, reduction in consciousness and dysautonomia. This temporal dichotomy was also seen in the timing of cerebrospinal fluid, electroencephalographic and in the rather infrequent cerebral imaging changes. Overall, our data support a model in which the early features are associated with cerebrospinal fluid lymphocytosis, and the later features with appearance of oligoclonal bands. The immunological events and neuronal mechanisms underlying these observations need to be explored further, but one possibility is that the early stage represents diffusion of serum antibodies into the cortical grey matter, whereas the later stage results from secondary expansion of the immunological repertoire within the intrathecal compartment acting on subcortical neurons. Four patients, who only had temporal lobe epilepsy without oligoclonal bands, may represent restriction to the first stage.

Dataset Information

Teenager with acute psychosis due to non-paraneoplastic anti-N-methyl-d-aspartate receptor encephalitis with a successful recovery: A case report.

Introduction

Case presentation

Discussion

Conclusion

Publications

Teenager with acute psychosis due to non-paraneoplastic anti-N-methyl-d-aspartate receptor encephalitis with a successful recovery: A case report.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets