Deficit of brain and skeletal muscle bioenergetics in progressive supranuclear palsy shown in vivo by phosphorus magnetic resonance spectroscopy

Brain and muscle energy metabolism was assessed in vivo in five patients wi th progressive supranuclear palsy (PSP) using phosphorous magnetic resonanc e spectroscopy (P-31 MRS). P-31 MRS disclosed a reduced phosphocreatine (PC r) and an increased calculated free adenosine diphosphate (ADP) in the occi pital lobes of all patients. In our patients with PSP, inorganic phosphate (Pi) was significantly increased and Mg2+ was reduced. In the gastrocnemius muscle, Pi at rest was increased in four patients, and the three patients who were able to perform an incremental exercise showed a rate of PCr post- exercise recovery slower than control subjects. Our findings show that mult isystemic deficit of energy metabolism occurs in PSP and suggest that it ma y play a role in the pathogenesis of this disorder.