Yu-ichi Noto, Sonoko Misawa, Masahiro Mori, Naoki Kawaguchi, Kazuaki Kanai, Kazumoto Shibuya, Sagiri Isose, Saiko Nasu, Yukari Sekiguchi, Minako Beppu, Shigeki Ohmori, Masanori Nakagawa, Satoshi Kuwabara
OBJECTIVES: To clarify whether patients with spinal muscular atrophy (SMA) or spinal and bulbar muscular atrophy (SBMA) suffer disabling muscle fatigue, and whether activity-dependent conduction block (ADCB) contributes to their fatigue. ADCB is usually caused by reduced safety factor for impulse transmission in demyelinating diseases, whereas markedly increased axonal branching associated with collateral sprouting may reduce the safety factor in chronic lower motor neuron disorders. METHODS: We assessed the fatigue severity scale (FSS) in 22 patients with SMA/SBMA, and in 100 disease controls (multiple sclerosis, myasthenia gravis, chronic inflammatory demyelinating polyneuropathy (CIDP), and axonal neuropathy)...
September 2013: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology