[CITATION][C] Infantile muscular atrophy a prospective study with particular reference to a slowly progressive variety
V DUBOWITZ - Brain, 1964 - academic.oup.com
V DUBOWITZ
Brain, 1964•academic.oup.comTHE clinical picture which is usually associated with the diagnosis of infantile progressive
spinal muscular atrophy is one of severe generalized weakness, starting in the first year of
life and progressing rapidly to ultimate death from pneumonia, often before the first birthday
and usually by the age of 6 years. This is the picture which was so vividly described in a
series of papers by Werdnig (1891, 1894) and Hoffmann (1893, 1897, 1900). This disease
still occurs today and its course has not been materially changed by the advent of antibiotics …
spinal muscular atrophy is one of severe generalized weakness, starting in the first year of
life and progressing rapidly to ultimate death from pneumonia, often before the first birthday
and usually by the age of 6 years. This is the picture which was so vividly described in a
series of papers by Werdnig (1891, 1894) and Hoffmann (1893, 1897, 1900). This disease
still occurs today and its course has not been materially changed by the advent of antibiotics …
THE clinical picture which is usually associated with the diagnosis of infantile progressive spinal muscular atrophy is one of severe generalized weakness, starting in the first year of life and progressing rapidly to ultimate death from pneumonia, often before the first birthday and usually by the age of 6 years. This is the picture which was so vividly described in a series of papers by Werdnig (1891, 1894) and Hoffmann (1893, 1897, 1900). This disease still occurs today and its course has not been materially changed by the advent of antibiotics.
However, not all cases of neurogenic muscular atrophy starting in infancy have this malignant course. Since this is not generally realized these patients are either given a bad prognosis which is not borne out by subsequent events, or are rnisdiagnosed as benign congenital hypotonia (amyotonia congenita) or progressive muscular dystrophy.
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