| Prospective study of spinal muscular atrophy before age 6 years. DCN/SMA Group. | |
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MedLine Citation:
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PMID: 8352849 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Spinal muscular atrophy (SMA) is a common neuromuscular disorder of childhood, associated with a high mortality rate during the first 2 years of life. Most practitioners expect patients with SMA to follow a progressive course with loss of muscle strength and function over 2-10 years. Counselling sessions with parents frequently emphasize the high mortality rate and risk for respiratory failure. The progressive nature of SMA has been attributed to the loss of motor neurons. Fifty-eight children, ages 6 years and younger, were examined between January, 1987, and April, 1992, as part of a large, multicenter collaborative study of SMA. Muscle function was evaluated at regular intervals using a standardized protocol that was demonstrated to be reliable. We determined a prevalence of 56% for tongue fasciculations, a prevalence of 22% for facial weakness, and persistent deep tendon reflexes in one patient. Improved motor function and acquired milestones during the study were documented. This work should contribute toward a better understanding of the natural history of SMA. |
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Authors:
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S T Iannaccone; R H Browne; F J Samaha; C R Buncher |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Pediatric neurology Volume: 9 ISSN: 0887-8994 ISO Abbreviation: Pediatr. Neurol. Publication Date: 1993 May-Jun |
Date Detail:
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Created Date: 1993-09-22 Completed Date: 1993-09-22 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8508183 Medline TA: Pediatr Neurol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 187-93 Citation Subset: IM |
Affiliation:
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Department of Neurology, University of Texas Southwestern Medical Center, Dallas 75235-8897. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Child Child, Preschool Female Humans Infant Male Motor Skills / physiology Muscle Contraction / physiology Neurologic Examination Prospective Studies Range of Motion, Articular / physiology Reflex, Stretch / physiology Spinal Muscular Atrophies of Childhood / diagnosis*, physiopathology Sucking Behavior / physiology |
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