ABSTRACT

Antonia Ceccarelli, Eytan Raz, and Matilde Inglese

Demyelinating and metabolic diseases of the brain include a heterogeneous and large group of acquired and inherited disorders affecting primarily the white matter (WM) of the brain whose classication and diagnostic workup is complex. Traditionally, demyelinating diseases are characterized by an acquired damage/loss of properly formed myelin, whereas metabolic disorders of the brain include diseases that present an inherited dysmyelination/hypomyelination resulting from an altered synthesis, developmental, or maintenance disorder of myelin sheets. A comprehensive classication of WM diseases of the central nervous system (CNS) is presented in Table 26.1.