AN IN-VIVO AND IN-VITRO H-1-MAGNETIC RESONANCE SPECTROSCOPY STUDY OF MDX MOUSE-BRAIN - ABNORMAL-DEVELOPMENT OR NEURAL NECROSIS

Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder prim arily affecting young boys, often causing mental retardation in additi on to the well-known progressive muscular weakness. Normal dystrophin expression is lacking in skeletal muscle and the central nervous syste m (CNS) of both DMD children and the mdx mouse model. The underlying b iochemical lesion causing mental impairment in DMD is unknown. H-1-mag netic resonance spectroscopy (H-1-MRS) detects choline-containing comp ounds, creatine and N-acetyl aspartate (NAA) in vivo. NAA is commonly used as a chemical marker for neurons, and a decline in NAA is thought to correlate with neuronal loss. Control mice were compared to mdx us ing a combination of in vivo and in vitro H-1-MRS methods to determine whether neural necrosis or developmental abnormalities occur in dystr ophic brain. NAA levels were normal in mdx brain compared to controls suggesting minor, if any, neuronal necrosis in dystrophic brain. In co ntrast, choline compounds and myo-inositol levels were increased, indi cative of gliosis or developmental abnormalities in dystrophic brain.