ABSTRACT
Maple Syrup Urine Disease (MSUD) is caused by a severe deficiency in the branched-chain ketoacid dehydrogenase complex activity. Patients MSUD accumulate the branched-chain amino acids leucine (Leu), isoleucine, valine in blood, and other tissues. Leu and/or their branched-chain α-keto acids are linked to neurological damage in MSUD. When immediately diagnosed and treated, patients develop normally. Inflammation in MSUD can elicit a metabolic decompensation crisis. There are few cases of pregnancy in MSUD women, and little is known about the effect of maternal hyperleucinemia on the neurodevelopment of their babies. During pregnancy, some intercurrences like maternal infection or inflammation may affect fetal development and are linked to neurologic diseases. Lipopolysaccharide is widely accepted as a model of maternal inflammation. We analyzed the effects of maternal hyperleucinemia and inflammation and the possible positive impact the use of ibuprofen in Wistar rats on a battery of physics (ear unfolding, hair growing, incisors eruption, eye-opening, and auditive channel opening) and neurological reflexes (palmar grasp, surface righting, negative geotaxis, air-righting, and auditory-startle response) maturation parameters in the offspring. Maternal hyperleucinemia and inflammation delayed some physical parameters and neurological reflexes, indicating that both situations may be harmful to fetuses, and ibuprofen reversed some settings.
