Hemiplegic-type ALS: clinicopathological features of two autopsied patients

Introduction

In amyotrophic lateral sclerosis (ALS), it remains elusive how the initial lesions develop and further extend in the upper and lower motor neuron (UMN and LMN) systems. Several studies of ALS using cell or animal models have demonstrated that the disease-related protein, transactive response DNA-binding protein 43 kDa (TDP-43), propagates in the nervous system by sequential cell-to-cell transmission and/or anterograde/retrograde axonal transport.1 However, it is often difficult to trace the trajectory of disease progression in living patients, or propagation of TDP-43 in human autopsied tissues. This is partly due to the diverse clinical phenotypes of ALS and the resulting inter-individual variations in both the rate and manner of disease progression.2

Among the variants of ALS, hemiplegic-type ALS is characterised by prominent asymmetry of the clinical symptoms throughout the illness.3 Here we report the clinicopathological features of two autopsy cases of hemiplegic-type ALS with special reference…

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