AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Amyotrophic Lateral Sclerosis (ALS)

Amyotrophic Lateral Sclerosis (ALS)

Introduction

Amyotrophic Lateral Sclerosis (ALS), commonly known as Lou Gehrig's disease, is a neurological disease that affects the motor neurons. There is no definitive test for the diagnosis of ALS, so it is one of exclusion, relying on signs and symptoms that pertain to dysfunction in both upper motor neurons (UMNs) and lower motor neurons (LMNs). (Lepore, and Maragakis, 2007) There is only one treatment regularly used for the treatment of ALS, but it is not a cure. In general, ALS is diagnosed later than other disease processes in part because of the vague symptoms it can generate and also the time it takes for the physician to rule out other causes. The ultimate cause of death in ALS patients is the loss of muscle strength to properly breathe. The potential for stem cell research in playing a role in ALS treatment lies in the possibility of regenerating dead or dying motor neurons in the hopes of regaining muscle function and control.

Symptoms and Treatment

Because both the UMN and the LMN degenerate, both UMN signs and LMN signs are observed on clinical exam. Typically, UMN signs are problems you would foresee with the loss of the normal inhibitory input the UMNs usually have on the LMNs. That would lead one to see a hyperactive state in the musculature, which is indeed the case. Specific findings related to UMN degeneration include hyperreflexia (an exaggerated response to reflex testing), increased tone (an inability to relax certain muscle groups), and weakness.

As opposed to the UMN, the LMN provides an excitatory component to the muscle groups so that a loss of LMN health leads to a different set of signs and symptoms. LMN signs include fasciculations (twitching of isolated muscle groups), atrophy, and weakness. A combination of both ...