BACKGROUND It has reached a consensus that patients with amyotrophic lateral sclerosis (ALS) could display cognitive impairment characterized by executive dysfunction or even dementia, but cognitive spectrum of Chinese patients with ALS still waits to be documented. METHODS A total of 106 incident patients with sporadic ALS were enrolled and comprehensive neuropsychological tests covering mem...

Most amyotrophic lateral sclerosis (ALS) patients show focal onset of upper and lower motor neuron signs and spread of symptoms to other regions or the other side clinically. Progression patterns of sporadic ALS are unclear. The aim of this study was to evaluate the pattern of respiratory deterioration in sporadic ALS according to the onset site by using respiratory function tests. Study partic...

BACKGROUND There is less data available regarding the characteristics of cognitive impairment in patients with amyotrophic lateral sclerosis (ALS) in a population-based series. METHODOLOGY Patients with ALS incident in Piemonte, Italy, between 2009 and 2011 underwent an extensive neuropsychological battery. Cognitive status was classified as follows: normal cognition, frontotemporal dementia ...

May is ALS Awareness Month, observed to raise awareness of and foster research for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease. ALS is a progressive, fatal, neurodegenerative disorder of upper and lower motor neurons. The cause of ALS is not known, and no cure exists. In October 2010, the Agency for Toxic Substances and Disease Registry (ATSDR) launched the congressi...

Amyotrophic Lateral Sclerosis (ALS) is unique among the neuromuscular diseases (NMD) in that it’s diagnosis is clinical, and cannot be made with genetic tests, biopsies, or imaging. Current ALS diagnostic criteria reflect what “the experts” believe ALS is, not necessarily what ALS really is. Consequently, some 10 to 20% of ALS patients do not meet current diagnostic criteria. Bayesian diagnosis...

May is ALS Awareness Month, observed to raise awareness of and foster research for amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease. ALS is a progressive, fatal, neurodegenerative disorder of upper and lower motor neurons. The cause of ALS is not known, and no cure exists. In October 2010, the Agency for Toxic Substances and Disease Registry (ATSDR) launched the congressi...

OBJECTIVE Mutations in the progranulin (PGRN) gene were recently described as the cause of ubiquitin positive frontotemporal dementia (FTD). Clinical and pathological overlap between amyotrophic lateral sclerosis (ALS) and FTD prompted us to screen PGRN in patients with ALS and ALS-FTD. METHODS The PGRN gene was sequenced in 272 cases of sporadic ALS, 40 cases of familial ALS and in 49 patien...

BACKGROUND It is difficult to longitudinally characterize cognitive impairment in amyotrophic lateral sclerosis (ALS) due to motor deficits, and existing instruments aren't comparable with assessments in other dementias. METHODS The ALS Brief Cognitive Assessment (ALS-BCA) was validated in 70 subjects (37 with ALS) who also underwent detailed neuropsychological analysis. Cognitive predictors ...

Although executive functions in sporadic non-demented amyotrophic lateral sclerosis (ALS) patients are mostly affected, it remains unclear whether executive measures can differentiate between patients with bulbar and spinal ALS forms. Thirty spinal and 18 bulbar-onset ALS patients (ALS-s and ALS-b, respectively) as well as 47 demographically related healthy controls were examined in executive p...

Amyotrophic Lateral Sclerosis (ALS) is a devastative neurodegenerative disease characterized by selective loss of motoneurons. While several breakthroughs have been made in identifying ALS genetic defects, the detailed molecular mechanisms are still unclear. These genetic defects involve in numerous biological processes, which converge to a common destiny: motoneuron degeneration. In addition, ...