title: Relationship between cervical cord 1H-magnetic resonance spectroscopy and clinoco-electromyographic profile in amyotrophic lateral sclerosis.
abstract: BACKGROUND
  Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by the loss of motor neurons, leading to limb paralysis and respiratory failure.
  
  
  METHODS
  C1-C3 cord (1) H-magnetic resonance spectroscopy ((1) H-MRS) was performed in 19 patients with ALS and 20 controls. N-acetylaspartate (NAA), choline-containing compounds, creatine plus phosphocreatine (Cr), and myo-Inositol (m-Ins) were measured. ALS functional rating scale-revised (ALSFRS) and forced vital capacity (FVC) were assessed. The rates of decline were calculated at 6 months before and after (1) H-MRS.
  
  
  RESULTS
  NAA/Cr and NAA/m-Ins were decreased significantly, and m-Ins/Cr was increased significantly in ALS patients compared with controls. NAA/Cr and NAA/m-Ins were correlated with ALSFRS and FVC and inversely linked to the decline rates. NAA/Cr, NAA/m-Ins, and m-Ins/Cr were altered markedly in 9 patients with denervation and neurogenic changes in both C2 paraspinal and upper limb muscles.
  
  
  CONCLUSIONS
  These metabolite ratios were associated with disease progression and ongoing denervation in neck and hand muscles. C1-C3 cord (1) H-MRS might reflect anterior horn cell damage causing neck/arm weakness and respiratory dysfunction in ALS patients.
authors.affiliation: Department of Neurology, Toho University Omori Medical Center, 6-11-1, Omorinishi, Otaku, Tokyo, 143-8541, Japan. keni@med.toho-u.ac.jp
authors.forename: Ken
authors.initials: K
authors.lastname: Ikeda
authors.forename: Kiyoko
authors.initials: K
authors.lastname: Murata
authors.forename: Yuji
authors.initials: Y
authors.lastname: Kawase
authors.forename: Kiyokazu
authors.initials: K
authors.lastname: Kawabe
authors.forename: Osamu
authors.initials: O
authors.lastname: Kano
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Yoshii
authors.forename: Takanori
authors.initials: T
authors.lastname: Takazawa
authors.forename: Takehisa
authors.initials: T
authors.lastname: Hirayama
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Iwasaki
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D002574:Cervical Vertebrae; D002794:Choline / Q000378:metabolism; D003401:Creatine / Q000378:metabolism; D018450:Disease Progression; D004576:Electromyography; D005260:Female; D006801:Humans; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology; D009046:Motor Neurons / Q000378:metabolism* / Q000473:pathology; D010725:Phosphocreatine / Q000378:metabolism; D012720:Severity of Illness Index; D013116:Spinal Cord / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology
chemical_list: D010725:Phosphocreatine; D003401:Creatine; D002794:Choline