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.




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