Disseminated encephalomyelitis in adults

Disseminated encephalomyelitis (DEM) is an inflammatory demyelinating disease that is common in children, but also appears in adults. It is often misdiagnosed as multiple sclerosis (MS) from which it differs in its clinical presentation, course of disease and prognosis. Some aspects of DEM overlap with neuromyelitis optica (NMO), another demyelination disease of CNS that was for a long time regarded as part of the MS spectrum, until discovery of the aquaporin-4 antibodies, claimed to be specific for NMO. The clinical symptoms of both may be similar, and their clinical courses may be monophasic or multiphasic, mild but also very aggressive. Neuroimaging in both diseases is characterized by large demyelinating lesions in the spinal cord extending over several segments, and/or in the brain often involving the locations of astrocytes water channels. Our cases of monophasic, multiphasic and recurrent DEM, invoking possible causative triggers, point to the conclusion that DEM has to be regarded as a separate disease; its similarities with NMO raise the expectations that other specific autoantibodies will be identified to explain DEM and its variations.