Chronic progressive leukoencephalopathy with systemic arteriosclerosis in young adults.
Clinicopathological studies of four cases of chronic progressive leukoencephalopathy with systemic arteriosclerosis are reported. Two patients were siblings. In all of our patients, the illness began in early adulthood (25-29 years of age), with gait and speech disturbance as the first symptoms. At about the same time, the patients complained of lumbago, caused by intervertebral disc hernia, and had a tendency toward baldness. They gradually developed disturbance of memory, dementia, spastic paraplegia, and positive pathological reflexes. After a few years, they could neither stand nor walk and became decerebrate. Blood pressures ranged from 120/60 to 140/80 mm Hg. Laboratory data were within normal limits, including serum cholesterol. EEG showed a generalized slowing. The illness ended in death after 9 years in one case and 3-4 years in the other three cases. A general autopsy was done in three cases and only the brain was autopsied in one case. The visceral organs and the brains were fixed in 10% formalin for macroscopic and histological studies. The general autopsies revealed arteriosclerosis in the small arteries of the heart, spleen, and kidneys in three cases and in addition, moderate arteriosclerosis of the coronary arteries in one. Remarkably uniform changes were found in the brains of all four cases, involving diffuse demyelination of the cerebral white matter, with some preservation of U-fibers, and small cystic and softening foci in the white matter and the basal ganglia. The cytoarchitecture of the cerebral cortex was well-preserved. The degeneration of the white matter was caused by arteriosclerotic changes of the small arteries: fibrous intimal proliferation and hyaline degeneration and splitting of the internal elastic membrane.(ABSTRACT TRUNCATED AT 250 WORDS)
D000328:Adult; D001161:Arteriosclerosis / Q000235:genetics / Q000473:pathology*; D001921:Brain / Q000473:pathology*; D003704:Dementia / Q000235:genetics / Q000473:pathology*; D003711:Demyelinating Diseases / Q000235:genetics / Q000473:pathology*; D006801:Humans; D008297:Male; D010375:Pedigree; D013577:Syndrome