CDC: U.S. Prevalence of ALS Was 5.2 Per 100,000 in 2015
MONDAY, Nov. 26, 2018 -- In 2015, the prevalence of amyotrophic lateral sclerosis (ALS) in the United States was 5.2 per 100,000 population, with 16,583 cases identified, according to research published in the Nov. 23 issue of the U.S. Centers for...
Zoe Butti, Shunmoogum A. Patten
Background: New imaging techniques suggest an involvement of the motor corpus callosum (CC) in amyotrophic lateral sclerosis (ALS) (Agosta et al., 2010; Unrath et al., 2011; Kassubek et al., 2012). Yet, a functional involvement can not easily be shown only by the means of magnetic resonance imaging or fibre tracking. Our goal was to demonstrate functional deficits of the motor CC in ALS using transcranial magnetic stimulation (TMS).
Publication date: February 2019Source: The Lancet Neurology, Volume 18, Issue 2Author(s): David R Beers, Stanley H AppelSummaryNeuroinflammation is a common pathological feature of many neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS), and is characterised by activated CNS microglia and astroglia, proinflammatory peripheral lymphocytes, and macrophages. Data from clinical studies show that multiple genetic mutations linked to ALS (eg, mutations in SOD1, TARDBP, and C9orf72) enhance this neuroinflammation, which provides compelling evidence for immune dysregulation in the pathogenesis of ALS. Transg...
We describe a 19-year-old woman who was admitted to our hospital because of rapidly progressive dysarthria and dysphagia, developing over the course of 3 months. She had no family history of neurological diseases including essential tremor and no consanguineous history. Her delivery was non-eventful. In elementary school, her school records were not so bad, but she was not able to get along with her classmates.
Abstract Differentiated tissue is particularly vulnerable to alterations in protein and organelle homeostasis. The essential protein VCP, mutated in hereditary inclusion body myopathy, amyotrophic lateral sclerosis and frontotemporal dementia, is critical for efficient clearance of misfolded proteins and damaged organelles in dividing cells, but its role in terminally differentiated tissue affected by disease mutations is less clear. To understand the relevance of VCP in differentiated tissue, we inactivated it in skeletal muscle of adult mice. Surprisingly, knockout muscle demonstrated a necrotic myopathy with in...
New research, led by Harvard scientists, identifies a novel potential therapeutic target for treating amyotrophic lateral sclerosis (ALS).