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

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 1H‐magnetic resonance spectroscopy (1H‐MRS) was performed in 19 patients with ALS and 20 controls....

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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
Subjects:
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - metabolism
Amyotrophic Lateral Sclerosis - pathology
Amyotrophic Lateral Sclerosis - physiopathology
anterior horn cell damage
cervical cord
Cervical Vertebrae
Choline - metabolism
Creatine - metabolism
Disease Progression
Electromyography
Female
Humans
Magnetic Resonance Spectroscopy
Male
Medical research
Middle Aged
Motor Cortex - metabolism
Motor Cortex - pathology
Motor Cortex - physiopathology
Motor Neurons - metabolism
Motor Neurons - pathology
Phosphocreatine - metabolism
Severity of Illness Index
Spectrum analysis
Spinal Cord - metabolism
Spinal Cord - pathology
Spinal Cord - physiopathology
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Summary: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 1H‐magnetic resonance spectroscopy (1H‐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 1H‐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 1H‐MRS might reflect anterior horn cell damage causing neck/arm weakness and respiratory dysfunction in ALS patients. Muscle Nerve, 2013
ISSN:0148-639X
1097-4598
DOI:10.1002/mus.23467