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Updated: Aug 12, 2026

Utility of Dissociated Intrinsic Hand Muscle Atrophy in the Diagnosis of Amyotrophic Lateral Sclerosis
Published on: March 4, 2014
[Clinical analysis of late infantile form of muscular atrophy]
Clinical analysis of 47 cases of late infantile spinal muscular atrophy is reported. In 25.5% of cases mothers reported weak fetal movements in pregnancy and low muscular tonus after birth, these signs predicted early manifestation of the disease. In 89,2% of cases the first signs of the disease appeared within the first 3 years of life. Delayed motor development in infancy was observed in 59.6%. Immobilization developed between the ages of 2 and 18 years, usually after 7 years of disease duration, and it was not correlated with the age of onset of the first signs of the disease. Mental development was normal in 81.5% of cases. Familial character of the disease was found in 51.1% of cases and in some of them intrafamilial variability was observed.
Clinical analysis of 47 cases of late infantile spinal muscular atrophy is reported. In 25.5% of cases mothers reported weak fetal movements in pregnancy and low muscular tonus after birth, these signs predicted early manifestation of the disease. In 89,2% of cases the first signs of the disease appeared within the first 3 years of life. Delayed motor development in infancy was observed in 59.6%. Immobilization developed between the ages of 2 and 18 years, usually after 7 years of disease duration, and it was not correlated with the age of onset of the first signs of the disease. Mental development was normal in 81.5% of cases. Familial character of the disease was found in 51.1% of cases and in some of them intrafamilial variability was observed.
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