Relationship between cervical cord super(1)H-magnetic resonance spectroscopy and clinoco-electromyographic profile in amyotrophic lateral sclerosis

• Published in: Muscle & nerve 2013-01, Vol.47 (1), p.61-67
• Main Authors: Ikeda, Ken, Murata, Kiyoko, Kawase, Yuji, Kawabe, Kiyokazu, Kano, Osamu, Yoshii, Yasuhiro, Takazawa, Takanori, Hirayama, Takehisa, Iwasaki, Yasuo
• Format: Article
• Language: English
• ISSN: 0148-639X; 1097-4598
• DOI: 10.1002/mus.23467

Introduction: 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 super(1)H-magnetic resonance spectroscopy ( super(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 super(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 super(1)H-MRS might reflect anterior horn cell damage causing neck/arm weakness and respiratory dysfunction in ALS patients. Muscle Nerve, 2013