Donepezil treatment mitigates cholinergic system alterations, oxidative stress, neuroinflammation and memory impairment induced by branched-chain amino acid administration in rats.

Maple Syrup Urine Disease (MSUD) is an inherited metabolic disorder biochemically characterized by tissue accumulation of leucine, isoleucine, and valine and their derivatives. Patients present with neurological disabilities and treatment is limited. Donepezil, a drug used for neurodegenerative disorders, has been shown to improve memory and counteract oxidative stress and inflammation.

Memantine Improves Memory and Neurochemical Damage in a Model of Maple Syrup Urine Disease.

Maple Syrup Urine Disease (MSUD) is a metabolic disease characterized by the accumulation of branched-chain amino acids (BCAA) in different tissues due to a deficit in the branched-chain alpha-ketoacid dehydrogenase complex. The most common symptoms are poor feeding, psychomotor delay, and neurological damage. However, dietary therapy is not effective.

Acute effects of intracerebroventricular administration of α-ketoisocaproic acid in young rats on inflammatory parameters.

Maple Syrup Urine Disease (MSUD) is an autosomal recessive inborn error of metabolism (IEM), responsible for the accumulation of the branched-chain amino acids (BCAA) leucine, isoleucine, and valine, in addition to their α-keto acids α-ketoisocaproic acid (KIC), α-keto-β-methylvaleric acid (KMV), and α-ketoisovaleric acid (KIV) in the plasma and urine of patients. This process occurs due to a partial or total blockage of the dehydrogenase enzyme activity of branched-chain α-keto acids. Oxidative stress and inflammation are conditions commonly observed on IEM, and the inflammatory response may play an essential role in the pathophysiology of MSUD.