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Amyotrophic Lateral Sclerosis | ALS

amyotrophic lateral sclerosis complex?

Significance of apparent diffusion coefficient measurement for the differential diagnosis of multiple system atrophy, progressive supranuclear palsy, and Parkinson’s disease: evaluation by 3.0-T MR imaging. Williams DR, Lees AJ. Progressive supranuclear palsy: clinicopathological concepts and diagnostic challenges. Familial aggregation of parkinsonism in progressive supranuclear palsy. Brain iron deposition fingerprints in Parkinson’s disease and progressive supranuclear palsy. 

Predictors of survival in a series of clinically diagnosed progressive supranuclear palsy patients. Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s syndrome and PSP-parkinsonism. Functional impairment in progressive supranuclear palsy. The midbrain to pons ratio: a simple and specific MRI sign of progressive supranuclear palsy. The phenotypic spectrum of progressive supranuclear palsy: a retrospective multicenter study of 100 definite cases. 

Lang AE. Treatment of progressive supranuclear palsy and corticobasal degeneration. Zampieri C, Di Fabio RP. Improvement of gaze control after balance and eye movement training in patients with progressive supranuclear palsy: a quasi-randomized controlled trial. Paviour DC, Thornton JS, Lees AJ, J√§ger HR. 

Diffusion-weighted magnetic resonance imaging differentiates Parkinsonian variant of multiple-system atrophy from progressive supranuclear palsy. 

Keywords: [“progressive”,”palsy”,”supranuclear”]
Source: https://www.medscape.com/answers/1154074-99796/what-is-parkinsonism–dementia–amyotrophic-lateral-sclerosis-complex-pdals

New therapy for amyotrophic lateral sclerosis successfully tested on mice

A team from Universit√© Laval and the CERVO Brain Research Centre has demonstrated the efficacy in mice of a new therapy that addresses the main manifestation of amyotrophic lateral sclerosis. The researchers developed an antibody that reduces the number of TDP-43 protein aggregates in the brains of mice with ALS, resulting in significant improvements in their cognitive and motor performance. Details of this breakthrough were recently published in the Journal of Clinical Investigation. Dr. Julien and his team had already demonstrated in previous work that TDP-43 protein was overexpressed in the spinal cords of people with ALS. 

This overexpression leads to the formation of TDP-43 aggregates in nerve cells and an exaggerated inflammatory response that increases the neurons’ vulnerability. In their most recent study, Professor Julien and his colleagues produced an antibody that targets the TDP-43 protein. They inserted the genetic material encoding this antibody into viruses that were then injected into the nervous system of mice producing TDP-43 aggregates. This breakthrough paves the way for the development of immunotherapies for ALS and frontotemporal dementias involving TDP-43 aggregates. 

Keywords: [“TDP-43″,”aggregates”,”Julien”]
Source: https://www.sciencedaily.com/releases/2019/02/190206104554.htm

KEGG PATHWAY: hsa05014

Amyotrophic lateral sclerosis is a progressive, lethal, degenerative disorder of motor neurons. The hallmark of this disease is the selective death of motor neurons in the brain and spinal cord, leading to paralysis of voluntary muscles. Mutant superoxide dismutase 1, as seen in some familial ALS cases, is unstable, forming aggregates in the motor neuron cytoplasm, axoplasm and mitochondria. Collectively, these mechanisms are predicted to disturb cellular homeostasis, ultimately triggering motor neuron death. Amyotrophic lateral sclerosis: proposed mechanisms and pathways to treatment. 

ALS: a disease of motor neurons and their nonneuronal neighbors. Programmed cell death in amyotrophic lateral sclerosis. From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS. Journal Reference Authors. Pathways to motor neuron degeneration in transgenic mouse models. 

ALS-linked mutant SOD1 induces ER stress- and ASK1-dependent motor neuron death by targeting Derlin-1. Oxidative Stress: a common denominator in the pathogenesis of amyotrophic lateral sclerosis. Superoxide dismutase mutations of familial amyotrophic lateral sclerosis and the oxidative inactivation of calcineurin. 

Keywords: [“motor”,”neuron”,”Amyotrophic”]
Source: https://www.genome.jp/dbget-bin/www_bget?hsa05014

ALS

ALS is a chronic disorder that causes a loss of control of voluntary muscles. The onset of symptoms in ALS usually occurs between the ages of 50 and 60, although symptoms can occur earlier. According to the ALS Association, every year about 6,400 people in the United States are diagnosed with ALS. They also estimate that around 20,000 Americans are currently living with the disorder. ALS affects people in all racial, social, and economic groups. 

The symptoms of ALS depend on what areas of the nervous system are affected. ALS damages the corticospinal tract and causes spastic limb weakness. Emotional lability can occur in all ALS sufferers, even those without dementia. Genetic tests may also be useful for people with a family history of ALS.As ALS progresses, it becomes more difficult to breathe and digest food. Family members should talk to people with ALS about their care. 

People with ALS may need support when making medical decisions. Approximately 20 percent of patients live with ALS for over five years. The most common cause of death from ALS is respiratory failure. 

Keywords: [“ALS”,”symptom”,”occur”]

Source:
https://www.healthline.com/health/amyotrophic-lateral-sclerosis

amyotrophic lateral sclerosis

It is the most common form of MND and accounts for 65% to 85% of all cases of MND. Amyotrophic lateral sclerosis results from lesions to the corticospinal tract and the anterior horn cells and produces the characteristic feature of tonic atrophy – brisk reflexes and fasciculations. A late onset, rapidly progressive and ultimately fatal neurological disorder, caused by the loss of motor neurons in the brain and spinal cordfamilial aggregation of ALS, with an age-dependent but high penetrance, is a major risk factor for ALSfamilial ALS is clinically and genetically heterogeneousthree genes and linkage to four additional gene loci have been identified so far and may either predominantly lead to ALS or cause multisystem neurodegeneration with ALS as an occasional symptom. This form of the disease includes Progressive Bulbar Palsy. Corticospinal tract degeneration in the absence of significant muscle wasting may be referred to as primary lateral sclerosis. 

Keywords: [“ALS”,”gene”,”progressive”]
Source: https://www.gpnotebook.co.uk/simplepage.cfm?ID=-268042240