Further observations on subacute necrotizing encephalomyelopathy in adults

The clinical and neuropathlogical aspects of four cases of subacute necrotizing encephalomyelopathy (SNEM) in adults, are described. In one, the disease was precipitated by a surgical procedure, and caused death; this resembled cases previously reported. In the other three, the disease was recognized as an incidental pathological finding, without apparent morbidity. In these, the pathologic lesions were few in number, but were characteristic in location and nature, most specifically in the preservation of neurons within the devasted tissues. The disease in adults is thought due to a less severe metabolic defect than that which causes death in infants, and may be more common. When extrinsic stress is superimposed, the disease may become severe and cause death as in the first case and those previously reported. This might be prevented by the prophylactic use of those therapeutic agents which have had a favorable effect in infants, particularly very high doses of thiamine. The prevelence of the disease in adults, including the incomplete cases included in this report, suggests that such prophylactic measures may be warranted when stress is anticipated, as with surgery.