Infantile Necrotizing Encephalomyelopathy (Leigh)

Abstract

SINCE Leigh's publication¹ in 1951, 32 cases have been reported of a peculiar fatal encephalomyelopathy, affecting children exclusively. The clinical syndrome consists of feeding difficulties, psychomotor retardation, and, later, disturbances in the state of consciousness, ophthalmoplegia, ataxia, and muscular weakness. Beginning insidiously, often the condition deteriorates rapidly, and the child dies from respiratory complications within a few months. At autopsy there is bilateral, symmetrical degeneration of the brain stem, but at times the spinal cord is also affected. Glial and mesodermal proliferation is present in these regions, but the neurons are often well preserved. Numerous dilated small vessels are a conspicuous finding. The condition resembles Wernicke's encephalopathy. The most remarkable differences from Wernicke's encephalopathy are the absence of pathological changes in the mamillary bodies in nearly all cases and involvement of the spinal cord. Cases have been reported from Europe,^1-10 The United States,^11-13 Canada,¹⁴ Japan,