Adult form of subactute necrotizing encephalomyelopathy.
Subacute necrotizing encephalomyelopathy (SNE) usually appears before the age of 2 years. Variability in its clinical presentation make antemortem diagnosis difficult. The morphologic diagnosis depends on the present of necrotizing lesions with a characteristic distribution within the CNS. We describe a patient with the uncommon adult form of SNE. The pathologic changes are those accepted as diagnostic of the more common infantile SNE, and permit a separation from Wernicke's encephalopathy. In the adult, the disease is thought to be due to a less severe thiamine-related metabolic defect than that of the infantile form. The infrequent clinical recognition in the adult form may be due in part to lack of medical awareness.
D001927:Brain Diseases / Q000209:etiology / Q000473:pathology*; D001933:Brain Stem / Q000473:pathology; D004678:Encephalomalacia / Q000473:pathology; D005260:Female; D006801:Humans; D008526:Medulla Oblongata / Q000473:pathology; D008636:Mesencephalon / Q000473:pathology; D008875:Middle Aged; D013116:Spinal Cord / Q000473:pathology; D013118:Spinal Cord Diseases / Q000209:etiology / Q000473:pathology*; D013577:Syndrome