Rare autoimmune and autoinflammatory neurologic disorders

This chapter provides a comprehensive overview of the prevalence, clinical features, pathogenesis, and pathophysiology of less common autoimmune and autoinflammatory disorders that primarily affect the nervous system—autoimmune encephalitis, central nervous system (CNS) vasculitis, Hashimoto’s encephalopathy, autoimmune epilepsy, Morvan syndrome, Issacs’ syndrome, neuromyelitis optica spectrum disorders, autoimmune optic neuritis, neurosarcoidosis, neuro-Behcet’s disease, CNS lupus, myasthenia gravis, chronic inflammatory demyelinating polyradiculoneuropathy, and Lambert-Eaton myasthenic syndrome. It summarizes currently used diagnostic approaches and available therapeutic options. It also highlights associated socioeconomic impact and emphasizes needs for further research, innovation, and investment to better manage these debilitating neurologic conditions.