Cardiomyopathy of Friedreich's Ataxia: Use of Mouse Models to Understand Human Disease and Guide Therapeutic Development.

Pediatr Cardiol. 2011 Mar 1. [Epub ahead of print]

Payne RM, Pride PM, Babbey CM.

Riley Heart Research Center, Wells Center for Pediatric Research, Indiana University School of Medicine, 1044 West Walnut, R4302, Indianapolis, IN, 46202, USA

Keywords: Friedreich's ataxia, heart, brain, severe cardiomyopathy, heart failure, frataxin, Animal models, to develop new therapeutic strategies.

Brain diffusion-weighted imaging in Friedreich's ataxia

Mov Disord. 2011 Mar 2. doi: 10.1002/mds.23518.

Rizzo G, Tonon C, Valentino ML, Manners D, Fortuna F, Gellera C, Pini A, Ghezzo A, Baruzzi A, Testa C, Malucelli E, Barbiroli B, Carelli V, Lodi R.

MR Spectroscopy Unit, Department of Internal Medicine, Aging and Nephrology, University of Bologna, Bologna, Italy; Department of Neurological Sciences, University of Bologna, Bologna, Italy.

"The extent of neurodegeneration in FRDA appears more extended than previously reported."

Keywords: Friedreich ataxia (FRDA), brain damage in FRDA, in vivo markers of neurodegeneration, diffusion-weighted imaging (DWI), Axial DW images, mean diffusivity (MD) maps, medulla, pons, inferior, middle and superior cerebellar peduncles (ICP, SCP, MCP), dentate nucleus, cerebellar white matter, thalamus, caudate, putamen, pallidus, pyramidal tracts at level of posterior limb of internal capsule (PLIC), optic radiations (OR), and corpus callosum, Histograms of MD, International Cooperative Ataxia Rating Scale (ICARS).