| Natural history of SMA IIIb: muscle strength decreases in a predictable sequence and magnitude. | |
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MedLine Citation:
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PMID: 18725590 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: To assess the natural progression of muscle weakness in spinal muscular atrophy (SMA) IIIb. METHODS: Ten patients with SMA IIIb were followed for at least 10 years. Age at disease onset varied between 9 and 18 years. Patients were initially seen 2 to 10 years after disease onset. They were evaluated at approximately 2, 5, 10, 15, and 20 years of disease duration depending on the timing of their initial visit after onset. Medical Research Council (MRC) scale was used with particular attention to proximal muscles. RESULTS: The MRC grade declined with years in all of the muscles. The decline was usually not more than by one MRC grade for each 5-year period. There were 5-10 year periods when some muscles appeared to remain stationary. The succession of weakness was first triceps, then biceps and deltoid for upper extremity muscles and first thigh adductors, then iliopsoas, then quadriceps femoris, then hamstrings, thigh abductors, and gluteus maximus for lower extremity muscles. There was a remarkable uniformity between patients in the MRC grade for each muscle at each stage: in the first 5 years of the disease, triceps, iliopsoas, thigh adductors, and quadriceps femoris were the muscles which had noticeable weakness. CONCLUSIONS: These findings show that strength in spinal muscular atrophy IIIb decreases over time, explaining the progressive functional loss. The sequence of weakness in the lower extremities suggests that the disease starts segmentally involving the upper lumbar segments of the medulla spinalis initially. The slowness of the deterioration may have implications for clinical trials. |
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Authors:
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Feza Deymeer; Piraye Serdaroglu; Yesim Parman; Mehves Poda |
Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't |
Journal Detail:
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Title: Neurology Volume: 71 ISSN: 1526-632X ISO Abbreviation: Neurology Publication Date: 2008 Aug |
Date Detail:
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Created Date: 2008-08-26 Completed Date: 2008-09-26 Revised Date: 2009-06-10 |
Medline Journal Info:
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Nlm Unique ID: 0401060 Medline TA: Neurology Country: United States |
Other Details:
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Languages: eng Pagination: 644-9 Citation Subset: AIM; IM |
Affiliation:
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Department of Neurology, Istanbul Medical Faculty, Istanbul University, Capa 34390, Istanbul, Turkey. deymeer@istanbul.edu.tr |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Adult Arm / innervation, physiopathology Child Child, Preschool Disease Progression Female Humans Leg / innervation, physiopathology Longitudinal Studies Lumbar Vertebrae Male Motor Neurons / pathology Muscle Strength / physiology Muscle Weakness / diagnosis*, physiopathology* Muscle, Skeletal / innervation, physiopathology* Spinal Cord / pathology, physiopathology Spinal Muscular Atrophies of Childhood / diagnosis*, physiopathology* |
| Comments/Corrections | |
Comment In:
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Neurology. 2009 Jun 9;72(23):2057-8; author reply 2058
[PMID:
19506238
]
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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