We report an autopsy case of respirator-assisted long-survival sporadic amyotrophic lateral sclerosis (ALS) with extensive involvement extending beyond the motor system. The involved areas included the globus pallidus, subthalamic nucleus, red nucleus, substantia nigra, Clarke's column, intermediolateral nucleus, Onuf's (Onufrowicz) nucleus, middle root zone of the posterior column, and the spinocerebellar tract. There was no impairment of ocular movements. Such sporadic ALS cases with extensive involvement extending beyond the motor system may constitute a distinct subgroup of this disease that differs from long-survival ALS (with or without respirator support) in which only the motor system is affected.