What Do I Do Now? Patients with neuroimmunological syndromes pose many clinical challenges for the attending physician. Even experienced clinicians occasionally arrive at the point where diagnostic, work-up, treatment, or prognostic thinking becomes blocked. Neuroimmunology is a practical and accessible guide for both common and not-so-common neuroimmunological syndromes. Comprised of a collection of thirty short case vignettes, the information is presented in an easily digestible format that consists of a discussion highlighting the topic in question as well as an answer and advice section. These discussions are followed by a brief "take home" message emphasizing the clinical, diagnostic, and therapeutic pearls of the case. Neuroimmunology is the go-to resource for primary care physicians, neurologists and subspecialists who encounter central and peripheral demyelinating diseases, vasculitis, and other immune system related neurological disorders.

Aaron E. Miller, MD, Medical Director, Corinne Goldsmith Dickinson Center for Multiple Sclerosis, Department of Neurology, Mount Sinai School of Medicine, New York, NY. Tracy M. DeAngelis, Assistant Professor of Neurology, Mount Sinai School of Medicine, New York, NY.

1. Multiple Sclerosis Multiple Sclerosis (MS), the most common cause of disability due to a neurological disease in young adults, can pose a challenging diagnosis. In this chapter, we summarize the typical symptomatology, radiographic and paraclinical findings in MS. In addition, we briefly review candidate differential diagnostic entities, the current diagnostic criteria, as well as therapeutic options. 2. Acute Disseminated Encephalomyelitis Acute disseminated encephalomyelitis (ADEM), a fulminant episode of demyelinating disease, which can be confused with a first episode of multiple sclerosis (MS), can present with transiently severe neurological deficits requiring hospitalization and support care. In this chapter, we review the important differential diagnostic and therapeutic considerations and how best to differentiate ADEM from an initial presentation of MS. 3. Optic Neuritis Optic neuritis (ON), is an ophthalmological syndrome commonly encountered in inflammatory and demyelinating diseases of the central nervous system. Here we review the cardinal clinical examination findings, the possible associated systemic diseases in which ON can manifest, and the recommended diagnostic evaluation and therapeutic measures. 4. Transverse Myelitis The differential diagnosis of transverse myelitis is lengthy and can present a daunting challenge to the evaluating physician. Emphasizing the critical importance of an initial exclusion of spinal cord compressive causes, we then present several clinical red flags, which can assist in narrowing the potential etiologies and discuss available therapeutic options. 5. Neuromyelitis Optica Neuromyelitis Optica (NMO), a chronic inflammatory, demyelinating autoimmune disorder of the central nervous system with a predilection for the optic nerves and spinal cord, has long been confused with classical multiple sclerosis. In this chapter, we review the important clinical and radiographic distinctions of NMO and NMO spectrum disorders, as well as a briefly summarize promising new concepts in pathophysiology and therapeutic approaches. 6. Pregnancy and Multiple Sclerosis The risk of MS relapses is lower during pregnancy but significantly higher in the postpartum period. There are several key management issues to address in women of childbearing age with multiple sclerosis who are pregnant or family planning. In this chapter, we review important therapeutic issues regarding peripartum and postpartum disease management, data regarding the prospect of breastfeeding, and the psychosocial support and counseling to help patients and their families navigate these decisions. 7. Progressive Multifocal Leukoencephalopathy and Immune Reconstitution Inflammatory Syndrome Progressive Multifocal Leukoencephalopathy (PML), an opportunistic viral infection of central nervous system caused by the JC virus, typically manifests in severely immunocompromised conditions, ranging from HIV/AIDs to lymphoproliferative malignancies to the consequence of immunosuppressant medications, such as natalizumab, a monoclonal antibody approved for the treatment of relapsing forms of MS. In this chapter, we discuss the typical symptomatology and radiographic findings of PML and how to distinguish it from those of MS. In addition, we review the management of PML in natalizumab-treated MS patients as well as the features of immune reconstitution inflammatory syndrone (IRIS), the potentially life threatening consequence of natalizumab withdrawal in patients with PML. 8. Neuroborreliosis Neuroborreliosis, an infection of the nervous system by the spirochete Borrelia burgdorferi, is a controversial entity both in its proper diagnosis and management. In this chapter, we review the common presentations of Lyme infection affecting the central and peripheral nervous systems, the utility of diagnostic screening and confirmatory tests, and the recommended course of antibiotic treatment. 9. HTLV Myelopathy Infection due to the Human T-Lymphocytic 1 (HTLV-1) virus can result in a myelopathy involving the thoracolumbar spinal cord, which typically manifests clinically as a spastic paraparesis, hence its alternative moniker tropical spastic paraparesis. In this chapter, we summarize the diagnostic approach to a patient with a spastic paraparesis and the key points, which assist in narrowing down the lengthy differential diagnosis to HTLV-1. 10. Varicella Zoster Virus Myelopathy Varicella zoster virus (VZV) myelopathy is a rare cause of infectious myelitis and can present in both immunocompetent as well as immunocompromised individuals. In this chapter, we review the two most common presentations of VZV myelopathy, the optimal diagnostic testing and current therapeutic approaches. 11. Anticardiolipin Antibody Syndrome There can be considerable overlap between the neurological sequelae of Antiphospholid antibody syndrome (ACAS) and multiple sclerosis, creating a frustrating diagnostic dilemna for clinicians. In this chapter, we review the common features and distinct clinical manifestations of ACAS as well as the diagnostic criteria for ACAS. In addition, we discuss the therapeutic consideration of antiplatelet or anticoagulant therapy. 12. Rheumatoid Arthritis Rheumatoid arthritis (RA), is a systemic inflammatory disease that is characterized principally by a polyarthritis, but can results in several neurologic complications involving both the central and peripheral nervous system. In addition, several immunotherapies used to treat RA have been associated with neurological complications. In this chapter, we review the characteristic neurological sequelae of RA as well as the possible neurological consequences of its therapeutic regimens. 13. Neurobehcet's disease Behcet's disease is a multisystemic, inflammatory, likely vasculitic, disease, which typically involving recurrent orogenital mucocutaneous ulcerations, in addition to arthritic, ophthalmologic, gastrointestinal as well as neurologic manifestations. In this chapter, we review the current concepts in pathophysiology and diagnostic criteria for Behcet's disease. In addition, we discuss the typical neurological presentations both inflammatory and vascular and their respective therapeutic approaches. 14. Sjogren's Syndrome Primary Sjogren's syndrome involves the cardinal siccal syndrome of xerostomia and xeroophthalmia. Neurological manifestations generally affect the peripheral nervous system in the form of neuropathy but can also target the central nervous system and overlap with demyelinating diseases such as multiple sclerosis and neuromyelitis optica. In this chapter, we review the systemic and neurologic fea…