Tremor in motor neuron disease may be central rather than peripheral in origin [PDF]
BACKGROUND AND PURPOSE: Motor neuron disease (MND) refers to a spectrum of degenerative diseases affecting motor neurons. Recent clinical and post-mortem observations have revealed considerable variability in the phenotype.
Balint, B +9 more
core +2 more sources
Amyotrophic lateral sclerosis and cerebellum
AbstractAmyotrophic lateral sclerosis (ALS) is a devastating, heterogeneous neurodegenerative neuromuscular disease that leads to a fatal outcome within 2–5 years, and yet, a precise nature of the association between its major phenotypes and the cerebellar role in ALS pathology remains unknown.
Kabiljo, R +3 more
openaire +4 more sources
Optineurin defects cause TDP43-pathology with autophagic vacuolar formation
We previously showed that optineurin (OPTN) mutations lead to the development of amyotrophic lateral sclerosis. The association between OPTN mutations and the pathogenesis of amyotrophic lateral sclerosis remains unclear.
Takashi Kurashige +11 more
doaj +1 more source
Letter to the editor: autoimmune pathogenic mechanisms in amyotrophic lateral sclerosis [PDF]
The innate immune system may affect the function and survival of motor neurons in ALS by at least three mechanisms. First, there is evidence to suggest that aggregates of mutant SOD1—which is derived from microglial and astroglial cells—activate ...
de Vincentiis, M. +5 more
core +1 more source
The Overlapping Genetics of Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two diseases that form a broad neurodegenerative continuum. Considerable effort has been made to unravel the genetics of these disorders, and, based on this work, it is now clear ...
Yevgeniya A. Abramzon +3 more
semanticscholar +1 more source
Levosimendan for amyotrophic lateral sclerosis [PDF]
Levosimendan is a drug developed and approved in the EU to treat severe heart failure by intravenous administration. Its principal pharmacological effect is to increase cardiac contractility by calcium sensitisation of troponin C. Because of the positive action of the drug on the neuromechanical efficiency and contractile function of the diaphragm in ...
Carvalho, Mamede, Swash, Michael
openaire +4 more sources
Comparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathways [PDF]
Amyotrophic lateral sclerosis (ALS) is a devastating neurological disease with no effective treatment available. An increasing number of genetic causes of ALS are being identified, but how these genetic defects lead to motor neuron degeneration and ...
Anink, Jasper J. +17 more
core +3 more sources
Synaptic actions of amyotrophic-lateral-sclerosis-associated G85R-SOD1 in the squid giant synapse [PDF]
© The Author(s), 2020. This article is distributed under the terms of the Creative Commons Attribution License. The definitive version was published in Song, Y.
Song, Yuyu
core +1 more source
The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP-43) has remained a central focus to understand the disease.
Terry R. Suk, Maxime W. C. Rousseaux
semanticscholar +1 more source
Pain in amyotrophic lateral sclerosis
In this review, we discuss different aspects of pain syndrome in patients with amyotrophic lateral sclerosis: etiology, incidence, pathophysiology and main clinical features.
Vladislav B. Voitenkov, E. V. Ekusheva
doaj +1 more source

