Biological Perspectives

Dowben, Jonathan S.; Kowalski, Peter; Keltner, Norman L.

doi:10.1111/ppc.12132

Cited by 8 publications

(10 citation statements)

References 2 publications

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“…NMDARs are heterotetrameric ionotropic receptors , composed of two GluN1 subunits and combinations of two GluN2 or GluN3 subunits . The subunit composition of the receptor depends upon brain location and drives receptor function; GluN2A and GluN2B subunits, for example, are commonly seen in the forebrain, compared to GluN2C in the cerebellum .…”

Section: Pathophysiologymentioning

confidence: 99%

Anti-NMDA-Receptor Encephalitis: From Bench to Clinic

Venkatesan

Adatia

2017

ACS Chem. Neurosci.

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NMDAR encephalitis is a common cause of autoimmune encephalitis, predominantly affecting young adults. Current data supports the idea that autoantibodies targeting NMDARs are responsible for disease pathogenesis. While these autoantibodies occur in the setting of underlying malignancy in approximately half of all patients, initiating factors for the autoimmune response in the remainder of patients are unclear. While there is increasing evidence supporting viral triggers such as herpes simplex encephalitis, this association and the mechanism of action have not yet been fully described. Although the majority of patients achieve good outcomes, those without an underlying tumor consistently show worse outcomes, prolonged recovery, and more frequent relapses. The cloning of patient-specific autoantibodies from affected individuals has raised important questions as to disease pathophysiology and clinical heterogeneity. Further advances in our understanding of this disease and underlying triggers are necessary to develop treatments which improve outcomes in patients presenting in the absence of tumors.

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Section: Pathophysiologymentioning

confidence: 99%

Anti-NMDA-Receptor Encephalitis: From Bench to Clinic

Venkatesan

Adatia

2017

ACS Chem. Neurosci.

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“…Treatment involves immunosuppression with steroids and intravenous administration [1, 4, 7, 9]. Many patients recover well after appropriate treatment [10].…”

Section: Discussionmentioning

confidence: 99%

Chronic Neuropsychological Sequelae in a Patient with Nontumorous Anti-NMDA-Receptor Encephalitis

Han

Koehl

Patel

et al. 2017

Case Reports in Neurological Medicine

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Anti-N-methyl-D-aspartate receptor encephalitis is a neurological, autoimmune disorder tightly conceptualized only as recently as the mid-2000s. It presents itself in a combination of psychiatric, neurological, and autonomic features. We observe a unique case with probable earlier episode (prior to the mid-2000s conceptualization of the disease) and a later relapse, accompanying a comprehensive neuropsychological profile tracked after the relapse and subsequent improvement. Neurocognitive findings revealed residual frontal deficits with mood changes even in the state after plasmapheresis. This case is the first to describe posttreatment cognition in anti-NMDAR encephalitis after probable serial autoimmune episodes.

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“…Anti-NMDA receptor antibody encephalitis is increasingly identified as an etiology for noninfectious encephalitis in children and adults [ 6 ]. A classic presentation of the condition would be acute onset psychosis/encephalopathy with associated orofacial dyskinesias, without clear precipitant or injury, leading to progressive decline and coma in some [ 7 ]. Tumor as a substrate for a paraneoplastic etiology is not uncommon [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

Hemiataxia: A Novel Presentation of Anti-NMDA Receptor Antibody Mediated Encephalitis in an Adolescent

Phillips

Jones

Callaghan

et al. 2017

Case Reports in Psychiatry

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Anti-NMDA receptor antibody associated encephalitis as a cause of new-onset neuropsychiatric manifestations in children and adults can represent a significant diagnostic challenge for clinicians. Clinical signs often include encephalopathy, new-onset psychosis, and movement phenomenon. Although orofacial dyskinesias were initially identified as a characteristic movement phenomenon in this type of encephalitis, an expanded range of abnormalities has recently been reported, including isolated ataxia. We report a case of isolated hemiataxia in a young adult with mild initial psychiatric manifestations. A personal and family history of preceding neuropsychiatric symptoms produced diagnostic confusion and resulted in a significant diagnostic and therapeutic delay. Our case confirms the unilateral movement manifestations that have been emphasized in recent reports. Additionally, it confirms the need for involvement of neurologic as well as psychiatric services in the evaluation of such cases and emphasizes the importance of the neurologic examination in presentations with an initial psychiatric predominance.

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Biological Perspectives

Abstract: Early diagnosis and aggressive treatment promote better outcomes. First-line treatment includes immunotherapy.

Cited by 8 publications

References 2 publications

Anti-NMDA-Receptor Encephalitis: From Bench to Clinic

Anti-NMDA-Receptor Encephalitis: From Bench to Clinic

Chronic Neuropsychological Sequelae in a Patient with Nontumorous Anti-NMDA-Receptor Encephalitis

Hemiataxia: A Novel Presentation of Anti-NMDA Receptor Antibody Mediated Encephalitis in an Adolescent

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