PT  - JOURNAL ARTICLE
AU  - Keisuke Suzuki
AU  - Masahisa Katsuno
AU  - Haruhiko Banno
AU  - Yu Takeuchi
AU  - Motoshi Kawashima
AU  - Noriaki Suga
AU  - Atsushi Hashizume
AU  - Tetsuo Hama
AU  - Kei Uchida
AU  - Fumitada Yamashita
AU  - Tomohiko Nakamura
AU  - Masaaki Hirayama
AU  - Fumiaki Tanaka
AU  - Gen Sobue
TI  - The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy
AID  - 10.1136/jnnp.2009.190462
DP  - 2010 May 01
TA  - Journal of Neurology, Neurosurgery & Psychiatry
PG  - 567--571
VI  - 81
IP  - 5
4099  - http://jnnp.bmj.com/content/81/5/567.short
4100  - http://jnnp.bmj.com/content/81/5/567.full
SO  - J Neurol Neurosurg Psychiatry2010 May 01; 81
AB  - 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.