Editorial Hope and foresight for your patients with ALS

The astrophysicist Stephen Hawking, arguably the most famous patient with amyotrophic lateral sclerosis (ALS) to date, died on March 14, 2018. He was exceptional in many respects, and so was his disease. Hawking's long survival after an ALS diagnosis (over 50 years) gave hope to other patients, and his case helped ALS specialists illustrate disease variability and prognostic uncertainty. When caring for people with a neurodegenerative disease that usually progresses rapidly, like ALS, prognosis is key to plan medical care and future needs.
Source: Lancet Neurology - Category: Neurology Authors: Tags: Editorial Source Type: research

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CONCLUSIONS: Ultrasound-guided botulinum toxin injections into the parotid and submandibular glands seem to be a safe and effective therapy for the treatment of drooling. Further long-term prospective studies with varying doses are warranted. PMID: 30772963 [PubMed - in process]
Source: The Israel Medical Association Journal - Category: General Medicine Tags: Isr Med Assoc J Source Type: research
AbstractHereditary spastic paraplegias (HSPs) are clinically and genetically heterogeneous neurodegenerative disorders. Numerous genes linked to HSPs, overlapping phenotypes between HSP subtypes and other neurodegenerative disorders and the HSPs ’ dual mode of inheritance (both dominant and recessive) make the genetic diagnosis of HSPs complex and difficult. Out of the original HSP cohort comprising 306 index cases (familial and isolated) who had been tested according to “traditional workflow/guidelines” by Multiplex Ligation-dependen t Probe Amplification (MLPA) and Sanger sequencing, 30 unrelated patien...
Source: Neurogenetics - Category: Genetics & Stem Cells Source Type: research
Amyotrophic lateral sclerosis (ALS) is a fatal and rapidly progressing neurodegenerative disease without effective treatment. The receptor for advanced glycation end products (RAGE) and the toll-like receptor ...
Source: Journal of Neuroinflammation - Category: Neurology Authors: Tags: Research Source Type: research
Condition:   Amyotrophic Lateral Sclerosis Intervention:   Drug: Gold Nanocrystals Sponsors:   Clene Nanomedicine;   University of Texas Southwestern Medical Center Not yet recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
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Source: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration - Category: Neurology Authors: Source Type: research
Corrigendum to "Driven to decay: Excitability and synaptic abnormalities in amyotrophic lateral sclerosis" [Brain Research Bulletin 140 (2018) 318-333]. Brain Res Bull. 2019 Mar;146:327 Authors: Fogarty MJ PMID: 30770039 [PubMed - in process]
Source: Brain Research Bulletin - Category: Neurology Authors: Tags: Brain Res Bull Source Type: research
The FDA this week sent a warning letter to a Beverly Hills, Calif.-based surgeon for their marketing of an unapproved implantable device, dubbed the Pocket Protector, which the surgeon claims can prevent and treat capsular contracture, or scar tissue tightening, during breast implant procedures. In its letter, the federal watchdog also accuses the surgeon, Dr. Mark Berman, of “significant deviations from the FDA’s quality system requirements and current good manufacturing practices,” including charges related to the sterility of implantable devices. “This is not the first time the FDA has notified D...
Source: Mass Device - Category: Medical Devices Authors: Tags: Cosmetic/Aesthetic Featured Food & Drug Administration (FDA) Stem Cells Source Type: news
AbstractThe authors set out to study the role of T1-weighted muscle MRI in the diagnostic phase of ALS, comparing images from ten patients and nine age-matched healthy controls (HCs). All subjects underwent MRI of 68 muscles in the hands, paraspinal regions and lower limbs; the images were semi-quantitatively scored. Atrophy was more frequent in muscles of ALS patients than HCs (p 
Source: Skeletal Radiology - Category: Radiology Source Type: research
Abstract Cellular adaption to nutrient stress is exquisitely regulated, and its dysregulation could underlie human diseases including neurodegeneration. C9orf72 is linked to the most common forms of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) as well as rare cases of other neurological disorders. Recent studies have implicated C9orf72 functions in the autophagy-lysosome pathway, but the exact roles of C9orf72 remain unclear. We found that C9orf72 is required for the lysosomal targeting and degradation of CARM1, which is an important epigenetic regulator of macroautophagy/autophagy and lip...
Source: Autophagy - Category: Cytology Authors: Tags: Autophagy Source Type: research
A G4C2 hexanucleotide repeat expansion in the noncoding region of C9orf72 is the major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis (c9FTD/ALS). Putative disease mechanisms underlyin...
Source: Molecular Neurodegeneration - Category: Neurology Authors: Tags: Research article Source Type: research
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