title: The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy.
abstract: 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.
authors: Department of Neurology, Nagoya University Graduate School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466-8550, Japan.
authors: Keisuke
authors: K
authors: Suzuki
authors: Masahisa
authors: M
authors: Katsuno
authors: Haruhiko
authors: H
authors: Banno
authors: Yu
authors: Y
authors: Takeuchi
authors: Motoshi
authors: M
authors: Kawashima
authors: Noriaki
authors: N
authors: Suga
authors: Atsushi
authors: A
authors: Hashizume
authors: Tetsuo
authors: T
authors: Hama
authors: Kei
authors: K
authors: Uchida
authors: Fumitada
authors: F
authors: Yamashita
authors: Tomohiko
authors: T
authors: Nakamura
authors: Masaaki
authors: M
authors: Hirayama
authors: Fumiaki
authors: F
authors: Tanaka
authors: Gen
authors: G
authors: Sobue
mesh_terms: D017668:Age of Onset; D000368:Aged; D015415:Biomarkers; D002452:Cell Count; D004247:DNA / Q000235:genetics; D018450:Disease Progression; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D018737:Hand Strength / Q000502:physiology; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology*; D018485:Muscle Fibers, Skeletal / Q000473:pathology*; D053580:Muscle Strength / Q000502:physiology; D020966:Muscular Disorders, Atrophic / Q000235:genetics / Q000473:pathology*; D009460:Neurologic Examination
chemical_list: D015415:Biomarkers; D004247:DNA