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The authors report 20 cases of infantile spinal amyotrophy, including 8 sporadic and 12 familial. There were 10 boys and 10 girls, the age of onset of the disease being between 1 and 5 years, with an average of 32 months. The clinical course is such that these forms may be easily distinguished from the classical Werdnig-Hoffman syndrome, by virtue of their later onset and their slower progression.

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