ALS, also known as Lou Gehrig's disease, named for the Yankee baseball great who died from it, affects nerve cells in the brain and spinal cord that control voluntary muscle movement. The nerve cells waste away or die, and can no longer send messages to muscles, eventually leading to muscle weakening, twitching and an inability to move the arms, legs and body. Onset is typically around age 50 and death often occurs within three to five years of diagnosis. Some 10 percent of cases are hereditary.
There is no cure for ALS and there is only one FDA-approved drug treatment, which has just a small effect in slowing disease progression and increasing survival.
Even though myelin loss has not previously been thought to occur in the gray matter, a region in the brain where neurons process information, the researchers in the new study found in ALS patients a significant loss of myelin in one of every three samples of human tissue taken from the brain's gray matter, suggesting that the oligodendrocytes were abnormal. It isn't clear if the oligodendrocytes that form this myelin in the gray matter play a different role than in white matter the region in the brain where signals are relayed.
The findings further suggest that clues to the treatment of other diseases long believed to be focused in the brain's gray matter such as Alzheimer's disease, Huntington's disease and Parkinson's disease may be informed by studies of diseases of the white matter, such as multiple sclerosis (MS). Bergles says ALS and MS researchers never really thought their diseases had much in common before.
Oligodendrocytes have been under intense scrutiny in MS, Bergles says. In MS, the disease over time can tr
|Contact: Stephanie Desmon|
Johns Hopkins Medicine