title: [Intermediate forms of infantile spinal amyotrophy. Apropos of 20 personal observations].
abstract: 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. Intermediate forms also differ from the Kugelberg-Welander pseudo-myopathic form in developing earlier and having a less benign clinical course. These features favour the existence of a series of intermediate forms situated between the two classical types, the definition and limitations of the infantile spinal amyotrophies being relatively imprecise.
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D004576:Electromyography; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D009132:Muscles / Q000473:pathology; D009133:Muscular Atrophy / Q000145:classification* / Q000235:genetics / Q000473:pathology; D009410:Nerve Degeneration; D011379:Prognosis; D013118:Spinal Cord Diseases; D013577:Syndrome
vernacular_title: Les formes "intermédiaires" de l'amyotrophie spinale infantile. A propos de vingt observations personnelles.