Subacute Necrotizing Encephalomyelopathy

Abstract

In 1951, Leigh¹ published a case report of a peculiar neuropathological disorder in an infant. The disease, which he called subacute necrotizing encephalomyelopathy, was characterized by (1) multiple, frequently symmetrical foci of necrosis with predilection for the periaqueductal and periventricular structures and the tegmentum of the brain stem, (2) breakdown of the interstitial tissue of the nerve parenchyma with relative preservation of the cell bodies of the neurons, and (3) the prominence of small blood vessels. Although Leigh suggested that the disorder might represent Wernicke's disease in infants, there has been no convincing proof as to the etiology of this disorder. Subsequently, Feigin and Wolf (1954),² Richter (1957),³ and Reye (1960)⁴ added several cases of a similar neuropathological disease. The case reported here is the first recorded instance of the disease in Japan. The importance of the associated pathology of the peripheral nerves, spinal roots, and