Amyotrophic lateral sclerosis: A disorder of the frontal faculties?

Amyotrophic lateral sclerosis (ALS) was traditionally regarded as a disorder of only the motor neurons and their connections to the brain.  Although Charcot, who described and coined the term ALS, proposed a central origin of ALS, is was Professor Andrew Eisen who set the cats amongst the pigeons by suggesting that ALS was primarily a disorder of the upper motor neurons, with motor neuron degeneration being a secondary event.  The discovery of the c9orf72 gene as a major genetic cause of ALS underscored Professor Eisens’ hypothesis, although other supportive evidence has also been reported.

 

In an upcoming issue Chio’s group re-affirms that importance of cognitive  dysfunction in a population based study of Italian ALS patients.  Importantly these findings corroborate earlier studies , and have clear pathophysiological and potential therapeutic implications for ALS.

 

Read more at http://jnnp.bmj.com/content/early/2014/04/25/jnnp-2013-307223.short?g=w_jnnp_ahead_tab

 

(Visited 130 times, 1 visits today)