[Multicystic encephalomalacia in an adult--a case report].
A case of multicystic encephalomalacia found in adult life was described. A 35-year-old man was admitted to our hospital with a chief complaint of unsteadiness. He had developed normally until he fell into the shock state induced by mismatch blood transfusion at the age of 15 months. Since then he has been mentally retarded moderately and had clumsiness of the skillful movement in the right hand. The other neurological abnormality was hyperreflexia only. Laboratory examination failed to disclose metabolic defect. Both CT scan and MRI demonstrated numerous cystic lesions of various size spreading over bilateral cerebral white matter partially involving the inner layer of the cortex. On the contrary basal ganglia, cerebellum and brainstem were completely spared. The diagnosis of MCE was made from (1) anoxic-ischemic episode in infancy, (2) static clinical picture and (3) characteristic distribution of cystic lesions. It is well known that MCE results from perinatal hypoxia, but it is a polyetiologic condition caused by various damages to immature brain of early infancy and usually results in severe psychomotor retardation. Nonetheless, it is intriguing in our case that marked discrepancy was found between morphological change and neurological deficit. It is probable that at the age of 15 months the myelination of major projecting fibers was almost completed, but sufficient plasticity was preserved in immature brain. As a result, the patient had the neurological deficit in the minimum degree in spite of severe morphological change.
Sunada
Y
Y
Nishimura
Y
Y
Motoyoshi
Y
Y
D000328:Adult; D001927:Brain Diseases / Q000209:etiology / Q000473:pathology*; D003560:Cysts / Q000209:etiology / Q000473:pathology*; D004678:Encephalomalacia / Q000209:etiology / Q000473:pathology*; D006801:Humans; D002534:Hypoxia, Brain / Q000150:complications; D008297:Male