Investigation Of The Cerebral Energy Status In Patients With Glutaric Aciduria Type I By P-31 Magnetic Resonance Spectroscopy
NEUROPEDIATRICS(2003)
摘要
In vivo phosphorus magnetic resonance spectroscopy (MRS) was used to investigate markers of the cerebral energy status in two patients with glutaric aciduria type I (GA-I). Besides an increased concentration of phosphomonoesters in one patient, no other significant alterations from controls were found. This might indicate increased resynthesis of dendritic processes secondary to preceding metabolic crises. In contrast to previous cell-culture studies, no cerebral depletion of phosphocreatine (PCr) was observed. In conclusion, a severe global and permanent depletion of cerebral energy supplies must be ruled out. The benefit of a permanent creatine substitution to stabilize mitochondrial energy metabolism seems thus questionable. However, as MRS was performed during stable clinical conditions, the possibility of a PCr decrease during acute metabolic crises cannot be assessed.
更多查看译文
关键词
energy metabolism, glutaric aciduria type I, glutaryl-CoA dehydrogenase deficiency, magnetic resonance spectroscopy
AI 理解论文
溯源树
样例
生成溯源树,研究论文发展脉络