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Intro; Preface; Contents; Chapter 1: Epidemiology of Neuropsychiatric Systemic Lupus Erythematosus; 1.1 Introduction; 1.2 Classification; 1.3 Demographic Features of NPSLE; 1.3.1 Prevalence; 1.3.2 Age of Onset and Ethnicity; 1.3.3 Risk Factors; 1.4 The Limitations of 1999 ACR Nomenclatures and Definitions; 1.5 NPSLE in Childhood; 1.6 Steroid Induced Psychosis; 1.7 Mortality; 1.8 Emerging Problems from the Epidemiological Studies; 1.9 Summary; References; Chapter 2: Genetics; 2.1 Introduction; 2.2 Genetics of Overall SLE; 2.2.1 Major Histocompatibility Complex (MHC) Region.
2.2.2 Type I Interferon Pathway and Nucleic Acid Response Genes2.2.3 Defective Clearance of Dying Cell Nucleic Acids; 2.2.4 Signaling Molecules in Immune System Cells; 2.3 Genetics of Neuropsychiatric SLE; 2.3.1 TREX1; 2.3.2 Other Candidate Genes; 2.4 Summary; References; Chapter 3: Immunopathology of Neuropsychiatric Systemic Lupus Erythematosus; 3.1 Introduction; 3.2 Autoantibodies Implicated in the Pathogenesis of NPSLE; 3.2.1 Anti-Phospholipid Antibodies; 3.2.2 Anti-Ribosomal P Antibodies; 3.2.3 Anti-NMDA Receptor NR2 Subunit Antibodies; 3.2.4 Anti-Sm Antibodies.
3.2.5 Anti-Neuronal Antibodies3.2.6 Other Autoantibodies; 3.3 Intrathecal Ig Production and Blood-Brain Barrier Damages in NPSLE; 3.3.1 Intrathecal Ig Production; 3.3.2 Blood-Brain Barrier Damages in NPSLE; 3.4 Roles of Complements and Microglia in the Pathogenesis of NPSLE; 3.4.1 Complements; 3.4.2 Microglia; 3.5 Summary; References; Chapter 4: Pathology of Neuropsychiatric Systemic Lupus Erythematosus; 4.1 Introduction; 4.2 Overall Characteristic Features in Pathology in NPSLE; 4.3 The Pathogenesis of Vasculopathy in NPSLE; 4.4 Diffuse Psychiatric/Neuropsychological Syndromes.
4.4.1 Acute Confusional State4.4.2 Non-ACS Diffuse NPSLE; 4.5 Neurologic Syndromes; 4.5.1 Cerebrovascular Disease and Reversible Focal Neurological Deficits; 4.5.2 Seizures; 4.5.3 Cranial Neuropathy; 4.5.4 Myelopathy; 4.5.5 Peripheral Neuropathy; 4.6 Other Pathological Features in NPSLE; 4.6.1 Choroid Plexus; 4.6.2 Microglia; 4.6.3 Perivenous Changes; 4.7 Summary; References; Chapter 5: Clinical Features; 5.1 Introduction; 5.2 Classification; 5.3 Case Definition and Clinical Significance; 5.3.1 Diffuse Psychiatric/Neuropsychological Syndromes; 5.3.1.1 Acute Confusional State.
5.3.1.2 Anxiety Disorder5.3.1.3 Cognitive Dysfunction; 5.3.1.4 Mood Disorder; 5.3.1.5 Psychosis; 5.3.2 Neurologic Syndromes of CNS; 5.3.2.1 Aseptic Meningitis; 5.3.2.2 Cerebrovascular Disease; 5.3.2.3 Demyelinating Syndrome.; 5.3.2.4 Headache; 5.3.2.5 Movement Disorder (Chorea); 5.3.2.6 Myelopathy; 5.3.2.7 Seizures and Seizure Disorders; 5.3.3 Neurologic Syndromes of PNS; 5.3.3.1 Neuropathy, Cranial; 5.3.3.2 Acute Inflammatory Demyelinating Polyradiculoneuropathy (Guillain-Barré Syndrome); 5.3.3.3 Autonomic Disorder; 5.3.3.4 Mononeuropathy (Single/Multiplex); 5.3.3.5 Myasthenia Gravis.
2.2.2 Type I Interferon Pathway and Nucleic Acid Response Genes2.2.3 Defective Clearance of Dying Cell Nucleic Acids; 2.2.4 Signaling Molecules in Immune System Cells; 2.3 Genetics of Neuropsychiatric SLE; 2.3.1 TREX1; 2.3.2 Other Candidate Genes; 2.4 Summary; References; Chapter 3: Immunopathology of Neuropsychiatric Systemic Lupus Erythematosus; 3.1 Introduction; 3.2 Autoantibodies Implicated in the Pathogenesis of NPSLE; 3.2.1 Anti-Phospholipid Antibodies; 3.2.2 Anti-Ribosomal P Antibodies; 3.2.3 Anti-NMDA Receptor NR2 Subunit Antibodies; 3.2.4 Anti-Sm Antibodies.
3.2.5 Anti-Neuronal Antibodies3.2.6 Other Autoantibodies; 3.3 Intrathecal Ig Production and Blood-Brain Barrier Damages in NPSLE; 3.3.1 Intrathecal Ig Production; 3.3.2 Blood-Brain Barrier Damages in NPSLE; 3.4 Roles of Complements and Microglia in the Pathogenesis of NPSLE; 3.4.1 Complements; 3.4.2 Microglia; 3.5 Summary; References; Chapter 4: Pathology of Neuropsychiatric Systemic Lupus Erythematosus; 4.1 Introduction; 4.2 Overall Characteristic Features in Pathology in NPSLE; 4.3 The Pathogenesis of Vasculopathy in NPSLE; 4.4 Diffuse Psychiatric/Neuropsychological Syndromes.
4.4.1 Acute Confusional State4.4.2 Non-ACS Diffuse NPSLE; 4.5 Neurologic Syndromes; 4.5.1 Cerebrovascular Disease and Reversible Focal Neurological Deficits; 4.5.2 Seizures; 4.5.3 Cranial Neuropathy; 4.5.4 Myelopathy; 4.5.5 Peripheral Neuropathy; 4.6 Other Pathological Features in NPSLE; 4.6.1 Choroid Plexus; 4.6.2 Microglia; 4.6.3 Perivenous Changes; 4.7 Summary; References; Chapter 5: Clinical Features; 5.1 Introduction; 5.2 Classification; 5.3 Case Definition and Clinical Significance; 5.3.1 Diffuse Psychiatric/Neuropsychological Syndromes; 5.3.1.1 Acute Confusional State.
5.3.1.2 Anxiety Disorder5.3.1.3 Cognitive Dysfunction; 5.3.1.4 Mood Disorder; 5.3.1.5 Psychosis; 5.3.2 Neurologic Syndromes of CNS; 5.3.2.1 Aseptic Meningitis; 5.3.2.2 Cerebrovascular Disease; 5.3.2.3 Demyelinating Syndrome.; 5.3.2.4 Headache; 5.3.2.5 Movement Disorder (Chorea); 5.3.2.6 Myelopathy; 5.3.2.7 Seizures and Seizure Disorders; 5.3.3 Neurologic Syndromes of PNS; 5.3.3.1 Neuropathy, Cranial; 5.3.3.2 Acute Inflammatory Demyelinating Polyradiculoneuropathy (Guillain-Barré Syndrome); 5.3.3.3 Autonomic Disorder; 5.3.3.4 Mononeuropathy (Single/Multiplex); 5.3.3.5 Myasthenia Gravis.