| The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy. | |
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MedLine Citation:
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PMID: 19965845 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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OBJECTIVE: Spinal and bulbar muscular atrophy (SBMA) is a lower motor neuron disease caused by the expansion of a trinucleotide CAG repeat in the androgen receptor (AR) gene. The fundamental histopathological finding of this disease is an extensive loss of lower motor neurons in the spinal cord and brainstem. It is, however, difficult to evaluate clinically the degree of motor neuron degeneration, which stresses the need for biomarkers to detect the remaining neuronal function. METHODS: The authors performed motor unit number estimation (MUNE) in 52 patients with SBMA, to investigate whether this method could be a potential biomarker of SBMA, and re-evaluated MUNE 1 year later in a subgroup of the patients. RESULTS: The number of functioning motor units was remarkably reduced in patients with SBMA compared with controls, and was correlated with both ipsilateral grip power and disease duration. A longitudinal analysis demonstrated a further reduction in motor units within 1 year. CONCLUSIONS: The results suggest that MUNE is an electrophysiological parameter that reflects the severity and progression of motor neuron degeneration in patients with SBMA. |
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Authors:
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Keisuke Suzuki; Masahisa Katsuno; Haruhiko Banno; Yu Takeuchi; Motoshi Kawashima; Noriaki Suga; Atsushi Hashizume; Tetsuo Hama; Kei Uchida; Fumitada Yamashita; Tomohiko Nakamura; Masaaki Hirayama; Fumiaki Tanaka; Gen Sobue |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't Date: 2009-12-03 |
Journal Detail:
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Title: Journal of neurology, neurosurgery, and psychiatry Volume: 81 ISSN: 1468-330X ISO Abbreviation: J. Neurol. Neurosurg. Psychiatr. Publication Date: 2010 May |
Date Detail:
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Created Date: 2010-05-12 Completed Date: 2010-05-25 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 2985191R Medline TA: J Neurol Neurosurg Psychiatry Country: England |
Other Details:
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Languages: eng Pagination: 567-71 Citation Subset: IM |
Affiliation:
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Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Age of Onset Aged Biological Markers Cell Count DNA / genetics Disease Progression Female Functional Laterality / physiology Hand Strength / physiology Humans Longitudinal Studies Male Middle Aged Motor Neurons / pathology* Muscle Fibers, Skeletal / pathology* Muscle Strength / physiology Muscular Disorders, Atrophic / genetics, pathology* Neurologic Examination |
| Chemical | |
Reg. No./Substance:
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0/Biological Markers; 9007-49-2/DNA |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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