Here we review a total of 17 patients, including 4 of our own, with young-adult-onset arteriosclerotic leukoencephalopathy, alopecia and lumbago without hypertension. All were Japanese and the illness seemed to be transmitted in an autosomal recessive fashion. The male to female ratio was 7.5:1. The age of onset usually ranged from 25 to 30 years. Acute lumbago, spondylosis deformans, diffuse baldness, and progressive motor and mental deterioration were common clinical features. Cerebral arteriosclerosis and white mater disease were demonstrated pathologically or radiologically. Although the pathogenesis has remained obscure, we think this is a new clinico-pathological entity distinct from the classic type of Binswanger’s disease.