Does Verbal Communication Impairment Affect Quality of Life in Amyotrophic Lateral Sclerosis Patients? The purpose of this study was to examine the selfperceived QOL in ALS patients. Literature will be presented on the incidence, prevalence, prognosis, diagnosis and management of ALS, QOL studies for ALS, the role of the multidisciplinary team, the impairments and dysfunction that ALS patients ...

Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease affecting the motor neurons of the brain and spinal cord causing progressive paralysis and death [1]. ALS patients develop progressive muscle weakness, atrophy, then paralysis and death within 3 to 5 years after the onset of the disease [1,2]. Close to 90% of all ALS cases are sporadic (SALS) while the other 10% represent famili...

Previously, we have reported amyotrophic lateral sclerosis (ALS) families with multiple mutations in major ALS-associated genes. These findings provided evidence for an oligogenic basis of ALS. In our present study, we screened a cohort of 755 sporadic ALS patients, 111 familial ALS patients (97 families), and 765 control subjects of Dutch descent for mutations in vesicle-associated membrane pr...

OBJECTIVE Affective lability is an important dimension of adult ADHD, associated with marked impairments and worse outcomes. A valid and quick tool to measure affective lability may therefore be of interest. METHOD In 187 adult ADHD patients, we explored psychometric properties of the Affective Lability Scale-Short Form (ALS-18 items). We analyzed the construct validity and the external valid...

Amyotrophic lateral sclerosis (ALS) is a progressive fatal multisystemic neurodegenerative disorder caused by preferential degeneration of upper and lower motor neurons. To further delineate the genetic architecture of the disease, we used comprehensive panel sequencing in a cohort of 80 German ALS patients. The panel covered 39 confirmed ALS genes and candidate genes, as well as 238 genes asso...

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease caused by degeneration of upper and lower motor neurons. To date, glycosylation patterns of glycoproteins in fluids of ALS patients have not been described. Moreover, the aberrant glycosylation related to the pathogenesis of other neurodegenerative diseases encouraged us to explore the glycome of ALS patient sera. We found...

IMPORTANCE High-prevalence foci of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia complex (PDC) exist in Japanese on the Kii Peninsula of Japan and in the Chamorros of Guam. Clinical and neuropathologic similarities suggest that the disease in these 2 populations may be related. Recent findings showed that some of the Kii Peninsula ALS cases had pathogenic C9orf72 repeat expansio...

Amyotrophic lateral sclerosis (ALS) is the most common of motor neuron diseases, affecting 4-6 individuals per 100,000 individuals worldwide. ALS is characterized by muscle weakness and atrophy caused by the degeneration of neurons located in the motor cortex, brain stem and spinal cord. This fatal disease generally has an adult onset and progresses over a three to five year period. While 10% o...

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that affects upper motor neurons (MNs) comprising the corticospinal tract and lower MNs arising from the brain stem nuclei and ventral roots of the spinal cord, leading to fatal paralysis. Currently, there are no effective therapies for ALS. Increasing evidence indicates that neuroinflammation plays an important role...

Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are devastating neurodegenerative disorders with clinical, genetic, and neuropathological overlap. Hexanucleotide (GGGGCC) repeat expansions in a noncoding region of C9ORF72 are the major genetic cause of FTD and ALS (c9FTD/ALS). The RNA structure of GGGGCC repeats renders these transcripts susceptible to an unconventional me...