Fasciculation frequency is a questionable biomarker for motor unit loss in amyotrophic lateral sclerosis
نویسندگان
چکیده
منابع مشابه
Amyotrophic lateral sclerosis: a dying motor unit?
INTRODUCTION ALS is a late-onset neurodegenerative disease of unknown etiology. Since it was first described (Charcot, 1874), several mechanisms, such as oxidative stress, glutamate excitotoxicity, glial cell pathology, or aberrant RNA metabolism (for review, see Vucic and Kiernan, 2009). Recently the evidence accumulates that mitochondrial damage and defective axonal transport may play a pivot...
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Mutations in the gene superoxide dismutase 1 (SOD1) are causative for familial forms of the neurodegenerative disease amyotrophic lateral sclerosis. When the first SOD1 mutations were identified they were postulated to give rise to amyotrophic lateral sclerosis through a loss of function mechanism, but experimental data soon showed that the disease arises from a--still unknown--toxic gain of fu...
متن کاملCystatin C: A Candidate Biomarker for Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurologic disease characterized by progressive motor neuron degeneration. Clinical disease management is hindered by both a lengthy diagnostic process and the absence of effective treatments. Reliable panels of diagnostic, surrogate, and prognostic biomarkers are needed to accelerate disease diagnosis and expedite drug development. The cysteine pr...
متن کاملMotor Unit Number Index (MUNIX) detects motor neuron loss in pre-symptomatic muscles in Amyotrophic Lateral Sclerosis.
OBJECTIVE Motor Unit Number Index (MUNIX) is a quantitative neurophysiological measure that provides an index of the number of lower motor neurons supplying a muscle. It reflects the loss of motor neurons in patients with Amyotrophic Lateral Sclerosis (ALS). However, it is unclear whether MUNIX also detects motor unit loss in strong, non-wasted muscles. METHODS Three centres measured MUNIX in...
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Behçet’s disease is a multisystem vasculitis. Its neurological involvement mostly includes parenchymal and non-parenchymal central nervous system manifestations. Peripheral nervous system presentations are rare. A 32-yr-old male patient who fulfilled the international study group criteria for Behçet’s disease, referred to our center with walking difficulty and repeated falling downs. Neurologi...
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ژورنال
عنوان ژورنال: Brain Communications
سال: 2020
ISSN: 2632-1297
DOI: 10.1093/braincomms/fcaa188