Nora Weiduschat1,
Xiangling Mao1, Jonathan Hupf2, Nicole Armstrong2,
Hiroshi Mitsumoto2, Dikoma C. Shungu1
1Radiology,
Weill Cornell Medical College, New York, NY, United States; 2Neurology,
Columbia University College of Physicians and Surgeons, New York, NY, United
States
Oxidative stress has been implicated in both sporadic and familial forms of amyotrophic lateral sclerosis (ALS), suggesting an inadequate antioxidant defense system, of which glutathione (GSH) is the most abundant and important component. While GSH deficiency has been documented and associated with cell pathology and survival in preclinical models of ALS, direct in vivo evidence in patients brain is lacking. In this study, 1H MRS was used to measure and compare in vivo levels of GSH in the motor cortex of ALS patients and matched healthy volunteers (HV), and found significant deficits of the antioxidant in the patient group.