Anti-N-Methyl-D-Aspartate Encephalitis as Paraneoplastic Manifestation of Germ-Cells Tumours: A Cases Report and Literature Review.

Claudia Geraldine Rita,Ángela Carrasco Sayalero,Adriano Jimenez Escrig,Israel Nieto Gañan

doi:10.1155/2019/4762937

Claudia Geraldine Rita, Ángela Carrasco Sayalero + Show 2 more

Open Access

https://doi.org/10.1155/2019/4762937

Copy DOI

Abstract

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is the most common form of autoimmune encephalitis, caused by the interaction between an antibody and its target, located on glutamate receptor type N-methyl-D-aspartate (NMDA) of neuronal surface. There is a wide spectrum of clinical features starting by a viral-like prodrome, followed by symptoms such as psychosis, aggressive behaviour, memory loss, seizures, movement disorders, and autonomic instability. Up to 50% of the affected young female patients have germ-cells tumours as ovarian teratoma, making it essential to establish an early diagnosis through detection of specific antibodies in serum and cerebrospinal fluid (CSF). This retrospective observational study was performed in patients whom positive anti-NMDA receptor antibodies have been tested, associated with clinical manifestations that suggest autoimmune encephalitis and a germ-cell tumour confirmed by pathology. Six patients have tested positive for anti-NMDA receptor antibodies associated with a germ-cell tumour and clinical manifestations of autoimmune encephalitis. Management includes aggressive immunosuppression and surgical removal.

Highlights

Autoimmune encephalitis constitutes a group of neuroinflammatory pathologies, characterized by psychiatric and neurological manifestations caused by the interaction between an antibody (Ab) and its target that can be intracellular or in the cell surface [1, 2]
This retrospective observational study was performed in patients whom positive anti-NMDA receptor antibodies have been tested, associated with clinical manifestations that suggest autoimmune encephalitis and a germ-cell tumour confirmed by pathology
Anti-N-methyl-D-aspartate (NMDA) antibodies are directed against the NMDA receptor located on neuronal surface, being a heterotetramer composed by two subunits of glutamate ionotropic receptor 1 (GluN1) and two subunits of glutamate ionotropic receptor 2 (GluN2) that acts as a postsynaptic excitatory ionotropic receptor

Summary

Introduction

Autoimmune encephalitis constitutes a group of neuroinflammatory pathologies, characterized by psychiatric and neurological manifestations caused by the interaction between an antibody (Ab) and its target that can be intracellular or in the cell surface [1, 2]. An important aspect of encephalitis mediated by anti-NMDA receptor antibodies is that those directed against the GluN1 subunit are those that result in specific and recognizable syndromes, while those directed against the GluN2 subunits are not associated with any specific syndrome and, in most cases, its clinical and pathogenic value is uncertain [3]. Anti-NMDA receptor encephalitis is the most common form of autoimmune encephalitis, reaching 1% of all admissions of young adults to an intensive care unit. Up to 50% of the affected young female patients have germ-cells tumours as ovarian teratoma [1]. This study reports six cases of anti-NMDA receptor encephalitis associated with a germ-cell tumour

Cases Presentation

Findings

Discussion

Conclusion