Clinicopathological studies of four cases of chronic progressive @entity62 with @entity4288 are reported. Two @entity1 were siblings. In all of our @entity1 , 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 @entity1 complained of lumbago, caused by intervertebral disc hernia, and had a tendency toward baldness. They gradually developed @entity2414 , @entity303 , @entity451 , 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 @entity165 . 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% @entity406 for macroscopic and histological studies. The general autopsies revealed @entity4288 in the small arteries of the heart, spleen, and kidneys in three cases and in addition, moderate @entity4288 of the coronary arteries in one. Remarkably uniform changes were found in the brains of all four cases, involving @entity17 , 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 @entity62 was caused by arteriosclerotic changes of the small arteries: fibrous intimal proliferation and @entity528 and splitting of the internal elastic membrane.(ABSTRACT TRUNCATED AT 250 WORDS)

Chronic progressive XXXX with @entity4288 in young adults.

@entity1 :: ('9606', 'Species') :: ['patients'] @entity17 :: ('MESH:D003711', 'Disease') :: ['diffuse demyelination of the cerebral white matter'] @entity2414 :: ('MESH:D008569', 'Disease') :: ['disturbance of memory'] @entity303 :: ('MESH:D003704', 'Disease') :: ['dementia'] @entity406 :: ('MESH:D005557', 'Chemical') :: ['formalin'] @entity165 :: ('MESH:D002784', 'Chemical') :: ['cholesterol'] @entity451 :: ('MESH:C536857', 'Disease') :: ['spastic paraplegia'] @entity62 :: ('MESH:D056784', 'Disease') :: ['degeneration of the white matter', 'leukoencephalopathy'] @entity528 :: ('MESH:D009410', 'Disease') :: ['hyaline degeneration'] @entity4288 :: ('MESH:D001161', 'Disease') :: ['arteriosclerosis', 'systemic arteriosclerosis']
@entity62 :: (MESH:D056784,Disease) :: ['leukoencephalopathy']
