Objective biomarkers for amyotrophic lateral sclerosis would facilitate the discovery of new treatments. The common neurotrophin receptor p75 is up regulated and the extracellular domain cleaved from injured neurons and peripheral glia in amyotrophic lateral sclerosis. We have tested the hypothesis that urinary levels of extracellular neurotrophin receptor p75 serve as a biomarker for both huma...

Amyotrophic lateral sclerosis is a rapidly progressing fatal neurodegenerative disease characterized by the presence of protein inclusions within affected motor neurons. Endoplasmic reticulum stress leading to apoptosis was recently recognized to be an important process in the pathogenesis of sporadic human amyotrophic lateral sclerosis as well as in transgenic models of mutant superoxide dismu...

Intronic expansion of the GGGGCC hexanucleotide repeat within the C9ORF72 gene causes frontotemporal dementia and amyotrophic lateral sclerosis/motor neuron disease in both familial and sporadic cases. Initial reports indicate that this variant within the frontotemporal dementia/amyotrophic lateral sclerosis spectrum is associated with transactive response DNA binding protein (TDP-43) proteinop...

Amyotrophic lateral sclerosis is a devastating neurodegenerative disorder characterized by the progressive loss of spinal motor neurons. While the aetiological mechanisms underlying the disease remain poorly understood, oxidative stress is a central component of amyotrophic lateral sclerosis and contributes to motor neuron injury. Recently, oxidation resistance 1 (OXR1) has emerged as a critica...

BACKGROUND Amyotrophic lateral sclerosis is a slowly progressive fetal neurodegenerative disease in which clinical phenotype and nutritional status are considered prognostic factors. Advanced age has also been reported to carry a poor prognosis in amyotrophic lateral sclerosis. The elderly population is expected to increase in Japan, as well as in other countries in the near future. Whether lat...

To investigate emotion recognition in amyotrophic lateral sclerosis (ALS) patients without cognitive/behavioral deficits and its relationship with the integrity of basal ganglia, hippocampus amygdala.

BACKGROUND Dietary exposure to the cyanotoxin BMAA is suspected to be the cause of amyotrophic lateral sclerosis in the Western Pacific Islands. In Europe and North America, this toxin has been identified in the marine environment of amyotrophic lateral sclerosis clusters but, to date, only few dietary exposures have been described. OBJECTIVES We aimed at identifying cluster(s) of amyotrophic...

Impairments in the ability to recognize and attribute emotional states to others have been described in amyotrophic lateral sclerosis patients and linked to the dysfunction of key nodes of the emotional empathy network. Microstructural correlates of such disorders are still unexplored. We investigated the white-matter substrates of emotional attribution deficits in a sample of amyotrophic later...

INTRODUCTION The cardiovascular consequences related to amyotrophic lateral sclerosis are relatively underappreciated. The disease invokes a systematic degeneration of autonomic neurons leading to autonomic dysfunction. We therefore hypothesized that patients with amyotrophic lateral sclerosis may have a predilection to the development of cardiac conduction disorders. CASE PRESENTATION A 65-y...