We report on 3 brothers with a myopathy that also affected their maternal grandfather and great-uncle. Characteristic features are onset in early childhood, very slow progression, normal life expectancy, weakness of proximal limb muscles, especially in the legs, elevation of serum creatine kinase, and no cardiac or intellectual involvement. In biopsy material muscle fibers are almost never necrotic but show excessive autophagic activity and exocytosis of the phagocytosed material. We suggest that this family has an undescribed type of congenital myopathy, for which we propose the name X-linked myopathy with excessive autophagy.

The effect of levodopa on the mortality of patients with Parkinson's disease was investigated in 349 patients treated with levodopa or levodopa combined with decarboxylase inhibitor during 1969-1975 inclusive. During the study period, 61 patients died. The expected mortality was 32.99 resulting in a ratio of actual to expected deaths of 1.85. The excess mortality was accounted for by patients with a severe disease at entry and especially, by the less favorable effect of levodopa treatment than in the living patients. In comparison with the prelevodopa era, the reduction of mortality and the increase of life expectancy of patients with Parkinson's disease during levodopa treatment possibly reflect the decrease of the early mortality due to Parkinson's disease.

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This paper summarizes the clinical and genetic features of a disease occurring in 16 patients from the same extended family, which resembles the multi-infarct dementia described by Sourander and Wålinder [Acta neuropath. 39:247-254,1977]. This family has relapsing strokes with neuropsychiatric symptoms, and they affect relatively young adult individuals of both sexes. The entity of the disease is characterized by autosomal dominant transmission with late onset and by association with occlusive cerebrovascular infarcts in the white matter, which was also generally reduced. Both of these features can be seen in the CT scan. In 13 members of this family the diagnosis can be regarded as certain and in a further 3 cases as more or less probable.