Head drop in progressive supranuclear palsy: An unusual association with amyotrophic lateral sclerosis

摘要

Progressive supranuclear palsy (PSP) is a sporadic, progressive atypical extrapyramidal disorder characterized by postural instability, supranuclear ophthalmoplegia, axial rigidity and mild dementia. Associations between motor neuron disease (ALS) and other neurodegenerative disorders such as frontotemporal dementia (FTD) have been extensively described, and the presence of abnormal intracellular accumulation of pathological TDP 43 or FUS proteins in both ALS and FTD suggests a common pathological background [1]. Despite this, the coexistence of PSP and amyotro-phic lateral sclerosis (ALS) remains unusual.