Cognitive impairment in amyotrophic lateral sclerosis (ALS) is correlated with pathologic and radiographic changes in cerebral cortex beyond the motor regions. Clinically, evidence of impairment can be detected in up…
Compromised nutrition leading to weight loss is a common and significant problem in the amyotrophic lateral sclerosis (ALS) patient population. The benefit of aggressive and early nutritional therapy can profoundly…
Although quality of life is difficult to define clearly, a number of instruments and questionnaires have been developed and applied to patients who have amyotrophic lateral sclerosis (ALS). This article…
Clinical trials in amyotrophic lateral sclerosis have significantly evolved over the last decade. New outcome measures have been developed that have reduced the sample size requirement as compared with survival…
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting the motor nervous system. It causes progressive and cumulative physical disabilities in patients, and leads to eventual death due to respiratory…
Motor unit number estimation (MUNE) is a unique electrophysiologic test used to estimate the number of surviving motor units in a muscle or group of muscles. It is used most…
Polyglutamine repeat expansion in the androgen receptor is responsible for the motor neuron degeneration in X-linked spinal and bulbar muscular atrophy (SBMA; Kennedy’s disease). This mutation, like the other polyglutamine…
New knowledge of the structure and function of the human genome and novel genomic technologies are being applied to the study of sporadic amyotrophic lateral sclerosis (ALS). These studies can…
The role of surgery in the management of dysphagia is clear in some areas and controversial in others. Evaluation for the causes of dysphagia can elucidate conditions in which surgery…
Amyotrophic lateral sclerosis (ALS) was first described by Charcot in 1869 as what we would now call a sporadic disease—a disease believed to occur without a strong genetic influence. Only…
