Guillain-Barré Syndrome

This chapter begins by outlining the origin of Guillain-Barré syndrome (GBS). It looks at the epidemiology and antecedent events. It also considers clinical manifestations. The classic clinical scenario is one of acute, symmetric, progressive, ascending weakness, and areflexia, with or without sensory symptoms days to weeks after a preceding infection. Next differential diagnosis is examined. The differential diagnosis of GBS is quite broad and includes disorders leading to sudden onset of weakness. The general pattern of clinical involvement and medical history helps to hone the differential. Finally, the chapter looks at management of the syndrome and treatment options for various different groups, such as immune therapy.

Neuroimmunology for the Non-Immunologist

The chapter begins with a short introduction to the components of the immune system, outlining both the innate and adaptive components. It discusses the role of the immune system in protecting against infections and abnormal tissues. It describes the concepts of self-antigens, antigen presentation, and immune synapse. It then examines immune tolerance and the differing functions and capacities of the innate and adaptive immune systems. Finally, the chapter considers infections and autoimmune phenomena and how the immune system responds to these challenges.

Neuromyelitis Optica Spectrum Disorders

Neuromyelitis optica spectrum disorders (NMOSD) are inflammatory diseases of the central nervous system, traditionally diagnosed in patients with inflammatory attacks restricted to the optic nerves and spinal cord. The chapter considers the epidemiology, pathophysiology and immunopathogenesis of NMOSD. The clinical presentation and radiographic features are reviewed and the prognosis of patients with NMOSD is considered. The mortality and morbidity of untreated NMOSD is much greater than those of MS. Treatment options are summarized and finally future prospects of research are considered.

Multiple Sclerosis

This chapter provides a broad overview of adult onset multiple sclerosis (MS), with the intent of providing a practical approach to diagnosis and treatments. It starts by looking at the epidemiology and demographics of MS, followed by a detailed discussion of the pathogenesis and pathology of MS. The chapter discusses the diagnosis, the various stages, and the symptoms of MS. Treatment of the disease, addressing both underlying pathology and symptoms, are discussed in detail. These include discussions of established therapies such as injectable and oral disease-modifying therapies, monoclonal antibodies, plasmapheresis or plasma exchange (PLEX), as well as new, and currently experimental therapies such as autologous stem cell transplants. Emphasis is especially directed at the often-underutilized management of MS-related symptoms.

Neurologic Complications of Systemic Autoimmune Diseases

This final chapter looks in detail at systemic inflammatory diseases that frequently affect both the central and peripheral nervous systems and can begin with neurological symptoms. These diseases cross traditional boundaries between neurology and rheumatology, and diagnosis and treatment require familiarity with the spectrum of neurological involvement with interdisciplinary communication. The chapter reviews some of the more common neurological manifestations of selected autoimmune diseases such as systemic lupus erythematosus, Sjögren syndrome, rheumatoid arthritis, scleroderma, sarcoidosis, primary angiitis of the central nervous system, systemic vasculitis syndromes, and antiphospholipid syndrome.

CIDP and Related Variants and Overlap Disorders

This chapter begins with a history of chronic immunological neuropathies. It then looks in particular at chronic inflammatory demyelinating polyneuropathy (CIDP), which is an immune-mediated peripheral nerve disorder characterized by progressive or relapsing motor or sensory symptoms. It then considers the epidemiology, clinical manifestations, and electrophysiology of CIDP. The chapter examines diagnostic data and diagnostic criteria for CIDP. It then looks at other neuropathies with clinical and electrophysiologic features that are shared with CIDP. Particular attention is given to neuropathy associated with monoclonal gammopathy including IgM associated neuropathy and POEMS syndrome, polyneuropathies associated with specific autoantibodies including antibodies that target nodal and paranodal structures, and multifocal motor neuropathy. For each condition diagnostic data, pathophysiology and treatment are discussed.

Immunopathogenesis of Inflammatory Myopathies

This chapter looks at inflammatory myopathies (IM), which constitute a heterogeneous group of acquired myopathies that have in common the presence of inflammation in the muscle tissue. The chapter looks at specific clinical features such as dermatomyositis, polymyositis, necrotizing autoimmune myositis, antisynthetase syndrome-overlap myositis, and inclusion body myositis. It then considers diagnosis, which can be made through muscle biopsy and the detection of autoantibodies. Finally, it looks at treatment options.

Paraneoplastic and Idiopathic Autoimmune Neurological Disorders Affecting the Central Nervous System and Autoimmune Dysautonomia

This chapter is an examination of immune-mediated central nervous system (CNS) disorders, which have increasingly been recognized as a critical disease category in the field of neurology. The chapter looks at clinical presentation, diagnostic evaluation, and treatment. The chapter also looks to the future. The field of immune-mediated neurological diseases is rapidly growing. New autoantibodies are being discovered at a rapid rate, helping unveil the mystery behind the challenging neurological presentations in many patients.

Immune-Mediated Disease of the Neuromuscular Junction

The chapter is concerned with disorders that affect the neuromuscular junction (NMJ). Myasthenia gravis (MG) is a prototypic antibody-mediated disease affecting the neuromuscular junction. The chapter looks at the epidemiology of MG. It also considers clinical manifestations and presentations, classification and staging, and diagnosis. The chapter also examines immunologic testing and issues such as pregnancy in MG. It considers future directions and treatment options in this area. Next, the chapter considers Lambert Eaton myasthenic syndrome (LEMS), which is a rare disease of the NMJ that is difficult to diagnose. The chapter looks at clinical manifestations, diagnosis, and treatment. Finally, the chapter briefly considers other immune-mediated diseases.