Amyotrophic lateral sclerosis with ophthalmoplegia and multisystem degeneration in patients on long-term use of respirators

摘要: We describe two patients with sporadic amyotrophic lateral sclerosis (ALS), who had developed progressive external ophthalmoplegia of a predominantly supranuclear type while they survived on respirators, and displayed histopathological abnormalities both typical atypical ALS. Patient 1 was 43-year-old man ALS 5-year duration, initially exhibited fulminant ALS, remained respirator for 4 years. 2 51-year-old 13-year 8 Both died in "totally locked-in state". Autopsy revealed not only consistent but also multisystem degeneration which involved the pontine tegmentum, substantia nigra, Clarke's dorsal nuclei spinocerebellar tracts. In addition, intracytoplasmic neuronal basophilic inclusion bodies marked immunoreactivity to anti-ubiquitin antibodies. Our case reports indicate that longer survival is possible through use respirators may make one subgroup prone develop clinical neuropathological features are observed during natural course