Autoimmune Encephalitis and Related Disorders of the Nervous System

Autoimmune encephalitis, diseases where the immune system attacks the brain, have become a fast-moving field of study in recent years. The authors, Drs Josep Dalmau and Francesc Graus, have played pivotal roles in the discovery of these diseases. Here, they provide a comprehensive clinical guide to the differential diagnosis of these disorders, illustrated with over 200 figures, 30 videos and numerous clinical vignettes, many from their own practice. Clinical descriptions are straightforward, emphasizing distinctive diagnostic clues for each disease. The strengths and weaknesses of diagnostic tests and clinical criteria are discussed extensively, as well as the best evidence supporting the use of available treatments. There is an up-to-date description of immunological triggers and comorbidities, and well-illustrated and clearly summarized pathogenic mechanisms and disease models. Gain full HTML access of the whole book, including supplementary videos, via a scratch-off code inside the cover.

• The authors, Josep Dalmau and Francesc Graus, have been at the forefront of the field over recent years and are perfectly positioned to write a comprehensive and practical reference guide to the differential diagnosis and treatment of autoimmune encephalitis.
• Covers the basic concepts of disease mechanisms and pathogenesis, providing a solid grounding in immunology and neurology.
• A Frequently Asked Questions chapter gives 90 example questions the authors have encountered and are answered briefly as a quick reference, with links to detailed information in the relevant chapters for in-depth guidance.
• Gain full HTML access of the whole book, with over 30 supplementary videos, via a scratch-off code inside the cover.
• Over 30 high quality videos and 200 figures illustrate the concepts covered in the book and help readers connect to the material along with numerous clinical vignettes that ground information in real world scenarios.

Contents
List of Clinical Vignettes viii
List of Videos x
Preface xi
List of Abbreviations xiii
Section 1 Overview
1 Importance, Definitions, History, Classification,
and Frequency of the Autoimmune
Encephalitides 1
2 General Approach to Diagnosis 19
3 Pathogenesis and Disease Mechanisms in
Neuronal Antibody-Mediated Encephalitis 42
Section 2 Antibodies and Antigens
4 Antibodies to Intracellular Antigens in CNS
Disorders 107
5 Antibodies to Neural Cell Surface Antigens 135
Section 3 Specific Syndromes and Diseases
6 Limbic Encephalitis 167
7 Autoimmunity Against Proteins Associated with
Voltage-Gated Potassium Channels 191
8 Anti-NMDAR Encephalitis 210
9 Seizures and Antibodies Against Surface
Antigens 255
10 Acute Disseminated Encephalomyelitis and Myelin
Oligodendrocyte Glycoprotein
Antibody-Associated Disease 290
11 Neuromyelitis Optica Spectrum Disorders and
Glial Fibrillary Acidic Protein Autoimmunity 315
12 Autoimmune Cerebellar Ataxias 342
13 Autoimmune Brainstem Encephalitis 368

14 Autoimmunity Against the Inhibitory
Synapsis 391
15 Anti-IgLON5 Disease 411
16 Autoimmune and Inflammatory
Encephalopathies as Complications of
Cancer 430
17 Deconstructing Hashimoto
Encephalopathy 460
18 CNS Syndromes at the Frontier of Autoimmune
Encephalitis 476
Section 4 Autoimmunity in Neurological
and Psychiatric Diseases
19 Autoimmune Psychosis 503
20 Psychiatric Manifestations of Autoimmune
Encephalitis 527
21 Abnormal Movements in Neurological
Autoimmune Disorders 545
22 Sleep and Autoimmunity 563
23 Immunity, Inflammation, and Epilepsy 588
24 Autoimmune Dementia: A Useful
Term? 611
25 Frequently Asked Questions on
Autoimmune Encephalitis and Related
Disorders 630
Index 656