Tumefactive Demyelination in Multiple Sclerosis: Clinical Presentation, Pathophysiology and Treatment Options

Tumefactive demyelinating lesions are caused by a heterogeneous group of central nervous system (CNS) idiopathic inflammatory demyelinating diseases. Tumefactive demyelinating lesions are uncommon and present a diagnostic challenge because they can be difficult to distinguish from tumors. This chapter reviews the clinical presentation, radiographic features, pathophysiology, prognosis, and management of patients with tumefactive demyelinating lesions, with specific attention to how these relate to typical multiple sclerosis. The rare variants of multiple sclerosis, including Schilder’s disease, Marburg acute multiple sclerosis, and Balo’s concentric sclerosis, are also discussed. Tumefactive presentations of neuromyelitis optic, acute disseminated encephalomyelitis (ADEM), and acute hemorrhagic leukoencephalitis are not covered in this chapter.