Researchers Pinpoint Cause of ALS

The actual cause of ALS, also known as Lou Gehrig’s disease, has long been elusive but new research out of Northwestern University Feinberg School of Medicine has pinpointed the cause of this deadly degenerative disease. If such is the case, the findings will improve chances of both quicker identification, as well as allow for development of treatment options.

ALS, technically known as amyotrophic lateral sclerosis, is a progressive neurodegenerative disease that causes motor neurons in the brain and spinal cord to die, leading to paralysis and death. ALS affects approximately 5 out of every 100,000 people worldwide.

According to a press release from Northwestern University, the new research found that the “basis of the disorder is a broken down protein recycling system in the neurons of the spinal cord and the brain. Optimal functioning of the neurons relies on efficient recycling of the protein building blocks in the cells. In ALS, that recycling system is broken. The cell can’t repair or maintain itself and becomes severely damaged.”

This dysfunctional recycling process linked to protein ubiquilin2 could be found in all versions of ALS.

There are two main types of ALS. Sporadic ALS and Familial ALS. The Sporadic version is the most common, occuring in approximately 90 percent of all ALS patients, some of which suffer from dementia as well. For approximately 25 percent of the sporadic group, ALS is preceded by Progressive Bulbar Palsy (PBP), which is centralized in the mouth and neck area and affects chewing and swallowing. PBP eventually leads to full-blown ALS.

Familial ALS occurs when a person inherits ALS genetics from a family member. This is much more rare, with an incidence rate of about 10 percent of all ALS cases. In addition there are several variant forms of ALS which effects a specific world region (Western Pacific ALS), age group (Juvenile ALS) or gender (Hiramaya Disease).

There has been no single effective treatment or cure for ALS, rather patients are treated for symptoms and disabilities occurring from the disease.