Topic: motor neuron disease

ALS is the most common motor neuron disease in humans, whereby upper and lower motor neurons degenerate. A major hypothesis underlying the mechanistic origin of neurodegeneration in ALS postulates that cortical hyperexcitability facilitates cell death. Previous research has identified the G4C2 hexanucleotide repeat expansion in the C9orf72 gene as the most common genetic cause of ALS; however, little is known about the contribution of the C9orf72 gene to neuronal excitability and synaptic dysfunction in the primary motor cortex. Thus, using C9orf72 knockout loss-of-function (C9-KO LOF) and gai

Amyotrophic lateral sclerosis (ALS) is a progressive motor neuron disease inexorably leading to premature death. Sleep disturbances have been ascribed to respiratory insufficiency, muscle cramps, spasticity, or restless legs syndrome, all leading to increased wakefulness. However, a recent neuropathological study in ALS patients described a loss of orexin-producing neurons, a neuropeptide involved in sleep and metabolic regulation, undermining the idea that sleep alterations are linked to central and peripheral changes. Yet, sleep alterations are poorly characterized in ALS, and their rela