Book contents
- Autoimmune Encephalitis and Related Disorders of the Nervous System
- Autoimmune Encephalitis and Related Disorders of the Nervous System
- Copyright page
- Dedication
- Contents
- Clinical Vignettes
- Videos
- Preface
- Abbreviations
- Section 1 Overview
- Section 2 Antibodies and Antigens
- Section 3 Specific Syndromes and Diseases
- Chapter 6 Limbic Encephalitis
- Chapter 7 Autoimmunity Against Proteins Associated with Voltage-Gated Potassium Channels
- Chapter 8 Anti-NMDAR Encephalitis
- Chapter 9 Seizures and Antibodies Against Surface Antigens
- Chapter 10 Acute Disseminated Encephalomyelitis and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease
- Chapter 11 Neuromyelitis Optica Spectrum Disorders and Glial Fibrillary Acidic Protein Autoimmunity
- Chapter 12 Autoimmune Cerebellar Ataxias
- Chapter 13 Autoimmune Brainstem Encephalitis
- Chapter 14 Autoimmunity Against the Inhibitory Synapsis
- Chapter 15 Anti-IgLON5 Disease
- Chapter 16 Autoimmune and Inflammatory Encephalopathies as Complications of Cancer
- Chapter 17 Deconstructing Hashimoto Encephalopathy
- Chapter 18 CNS Syndromes at the Frontier of Autoimmune Encephalitis
- Section 4 Autoimmunity in Neurological and Psychiatric Diseases
- Index
- References
Chapter 8 - Anti-NMDAR Encephalitis
from Section 3 - Specific Syndromes and Diseases
Published online by Cambridge University Press: 27 January 2022
- Autoimmune Encephalitis and Related Disorders of the Nervous System
- Autoimmune Encephalitis and Related Disorders of the Nervous System
- Copyright page
- Dedication
- Contents
- Clinical Vignettes
- Videos
- Preface
- Abbreviations
- Section 1 Overview
- Section 2 Antibodies and Antigens
- Section 3 Specific Syndromes and Diseases
- Chapter 6 Limbic Encephalitis
- Chapter 7 Autoimmunity Against Proteins Associated with Voltage-Gated Potassium Channels
- Chapter 8 Anti-NMDAR Encephalitis
- Chapter 9 Seizures and Antibodies Against Surface Antigens
- Chapter 10 Acute Disseminated Encephalomyelitis and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease
- Chapter 11 Neuromyelitis Optica Spectrum Disorders and Glial Fibrillary Acidic Protein Autoimmunity
- Chapter 12 Autoimmune Cerebellar Ataxias
- Chapter 13 Autoimmune Brainstem Encephalitis
- Chapter 14 Autoimmunity Against the Inhibitory Synapsis
- Chapter 15 Anti-IgLON5 Disease
- Chapter 16 Autoimmune and Inflammatory Encephalopathies as Complications of Cancer
- Chapter 17 Deconstructing Hashimoto Encephalopathy
- Chapter 18 CNS Syndromes at the Frontier of Autoimmune Encephalitis
- Section 4 Autoimmunity in Neurological and Psychiatric Diseases
- Index
- References
Summary
Anti-NMDAR encephalitis is the most frequent autoimmune encephalitis. It predominantly occurs in children and young females. Up to 80% of patients present with severe insomnia and psychiatric and behavioural symptoms that resemble those of psychotic episodes caused by primary psychiatric diseases. In addition to the psychiatric manifestations, patients develop neurological symptoms including seizures, abnormal movements, reduced verbal output, and dysautonomic features. Up to 50% of young females have an underlying ovarian teratoma that contains nervous tissue and NMDAR, which probably trigger the immune response. Less frequently, the encephalitis is triggered by an episode of herpes simplex encephalitis probably through the release of antigens by neurons damaged by the virus. The diagnosis of anti-NMDAR encephalitis requires the demonstration of the antibodies in CSF. Up to 14% of patients do not have detectable antibodies in serum. A positive result in serum but negative in CSF must be taken with caution as these patients do not present clinical features of encephalitis and many represent false positive results. Between 80% and 90% of patients respond to treatment which includes immunotherapy and removal of the tumour when it applies.
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- Publisher: Cambridge University PressPrint publication year: 2022
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