Abstract
Background: Tau-negative frontotemporal lober degeneration and amyotrophic lateral sclerosis (ALS) share the common neuropathology characterized by the deposition of TAR-DNA binding protein (TDP-43)-positive protein inclusions. It has been noticed that some ALS patients have dementia. Recent studies showed that more ALS patients had cognitive and behavior impairments. Pathologically, the cytoplasmic aggregation of TDP-43 is not restricted in the motor neurons but widespread throughout the brain in ALS patients.
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