| A collaborative study on the natural history of childhood and juvenile onset proximal spinal muscular atrophy (type II and III SMA): 569 patients. | |
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MedLine Citation:
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PMID: 9077498 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We analyzed clinical data of 569 patients in two combined series with childhood and juvenile proximal SMA. This cohort included only patients who had achieved the ability to sit unaided (type II and III SMA). The survival rate among 240 type II patients (who sat but never walked) was 98.5% at 5 years and 68.5% at 25 years. SMA III (n = 329) (those who walked and had symptoms before age 30 years) was subdivided into those with an onset before and after age 3 years (type IIIa, n = 195; SMA IIIb, n = 134). In patients with SMA III, life expectancy is not significantly less than a normal population. The probabilities of being able to walk at 10 years after onset was 70.3%, and at 40 years, 22.0% in SMA IIa. For SMA IIIb, 96.7% were walking 10 years after onset and 58.7% at 40 years. The subdivision of type III SMA was justified by the probability of being ambulatory depending on age at onset; the prognosis differed for those with onset before or after age 3 years. The data provide a reliable basis of the natural history of proximal SMA and support a classification system that is based primarily on age at onset and the achievement of motor milestones. |
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Authors:
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K Zerres; S Rudnik-Schöneborn; E Forrest; A Lusakowska; J Borkowska; I Hausmanowa-Petrusewicz |
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Publication Detail:
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Type: Clinical Trial; Controlled Clinical Trial; Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Journal of the neurological sciences Volume: 146 ISSN: 0022-510X ISO Abbreviation: J. Neurol. Sci. Publication Date: 1997 Feb |
Date Detail:
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Created Date: 1997-06-19 Completed Date: 1997-06-19 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 0375403 Medline TA: J Neurol Sci Country: NETHERLANDS |
Other Details:
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Languages: eng Pagination: 67-72 Citation Subset: IM |
Affiliation:
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Institute for Human Genetics, University of Bonn, Germany. zerres@snphysio2.wilhelm.uni-bonn.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Age of Onset Child Child, Preschool Databases, Factual* Disease Progression Female Germany Humans Infant Infant, Newborn Male Muscular Atrophy, Spinal / pathology, physiopathology* Poland Probability Spinal Muscular Atrophies of Childhood / pathology, physiopathology* Walking / physiology |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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