An autopsy case of primary lateral sclerosis with Alzheimer's disease

Primary lateral sclerosis (PLS) is a rare type of motor neuron disease (MND) characterized by the loss of upper motor neurons without lower motor neuron involvement [1]. In contrast, amyotrophic lateral sclerosis (ALS) is a common type of MND that is occasionally complicated by frontotemporal dementia (FTD) as the TDP-43 proteinopathy. However, there are few studies on the relationship of PLS with ALS, FTD and TDP-43 proteinopathy because of the rarity of autopsy cases. Here, we report a 68-year-old woman who exhibited typical clinical symptoms of PLS at onset, which then progressed to FTD, although her pathological diagnosis was PLS complicated by Alzheimer's disease (AD) pathology.

Source: Journal of the Neurological Sciences - March 19, 2020 Category: Neurology Authors: Takumi Nakamura, Tomoya Kon, Takeshi Kawarabayashi, Koichi Wakabayashi, Yoshio Ikeda, Mikio Shoji Tags: Letter to the Editor Source Type: research