Document Detail

The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy.
MedLine Citation:
PMID:  19965845     Owner:  NLM     Status:  MEDLINE    
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.
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:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2009-12-03
Journal Detail:
Title:  Journal of neurology, neurosurgery, and psychiatry     Volume:  81     ISSN:  1468-330X     ISO Abbreviation:  J. Neurol. Neurosurg. Psychiatr.     Publication Date:  2010 May 
Date Detail:
Created Date:  2010-05-12     Completed Date:  2010-05-25     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  2985191R     Medline TA:  J Neurol Neurosurg Psychiatry     Country:  England    
Other Details:
Languages:  eng     Pagination:  567-71     Citation Subset:  IM    
Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan.
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MeSH Terms
Age of Onset
Biological Markers
Cell Count
DNA / genetics
Disease Progression
Functional Laterality / physiology
Hand Strength / physiology
Longitudinal Studies
Middle Aged
Motor Neurons / pathology*
Muscle Fibers, Skeletal / pathology*
Muscle Strength / physiology
Muscular Disorders, Atrophic / genetics,  pathology*
Neurologic Examination
Reg. No./Substance:
0/Biological Markers; 9007-49-2/DNA

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