Combined Cerebellar Proton MR Spectroscopy and DWI Study of Patients with Friedreich's Ataxia

The Cerebellum
Laura Ludovica GramegnaR Lodi

Abstract

Friedreich's ataxia (FRDA) is the commonest autosomal recessive ataxia, caused by GAA triplet expansion in the frataxin gene. Neuropathological studies in FRDA demonstrate that besides the primary neurodegeneration of the dorsal root ganglia, there is a progressive atrophy of the cerebellar dentate nucleus. Diffusion-weighted imaging (DWI) detected microstructural alterations in the cerebellum of FRDA patients. To investigate the biochemical basis of these alterations, we used both DWI and proton MR spectroscopy (1H-MRS) to study the same cerebellar volume of interest (VOI) including the dentate nucleus. DWI and 1H-MRS study of the left cerebellar hemisphere was performed in 28 genetically proven FRDA patients and 35 healthy controls. In FRDA mean diffusivity (MD) values were calculated for the same 1H-MRS VOI. Clinical severity was evaluated using the International Cooperative Ataxia Rating Scale (ICARS). FRDA patients showed a significant reduction of N-acetyl-aspartate (NAA), a neuroaxonal marker, and choline (Cho), a membrane marker, both expressed relatively to creatine (Cr), and increased MD values. In FRDA patients NAA/Cr negatively correlated with MD values (r = -0.396, p = 0.037) and with ICARS score (r = -0.669, p < 0...Continue Reading

References

May 1, 1995·Journal of Computer Assisted Tomography·R A MoatsB D Ross
Dec 1, 1993·Magnetic Resonance in Medicine : Official Journal of the Society of Magnetic Resonance in Medicine·S W Provencher
Oct 17, 1996·The New England Journal of Medicine·A DürrM Koenig
Jul 13, 1999·Annals of Neurology·D WaldvogelM Hallett
Sep 29, 1999·Proceedings of the National Academy of Sciences of the United States of America·R LodiA H Schapira
Dec 23, 1999·Human Molecular Genetics·J L BradleyA H Schapira
Sep 7, 2000·Magnetic Resonance in Medicine : Official Journal of the Society of Magnetic Resonance in Medicine·M A McLeanJ S Duncan
May 15, 2001·Biological Signals and Receptors·R LodiA H Schapira
Jun 19, 2001·NMR in Biomedicine·S W Provencher
May 18, 2005·Journal of the Neurological Sciences·Vittorio CalabreseD Allan Butterfield
Jun 21, 2005·Brain Research·Martin ViauYvan Boulanger
May 9, 2006·Antioxidants & Redox Signaling·Raffaele LodiAnthony H V Schapira
Apr 20, 2007·Acta Neuropathologica·Arnulf H KoeppenJacques B Lamarche
Jul 20, 2007·Journal of Neurology, Neurosurgery, and Psychiatry·R Della NaveM Mascalchi
Aug 20, 2008·Expert Opinion on Pharmacotherapy·Caterina Tonon, Raffaele Lodi
Oct 22, 2008·Brain : a Journal of Neurology·Filippo FortunaValerio Carelli
Mar 13, 2009·Journal of Neurology·Marcondes C FrançaFernando Cendes
Apr 11, 2009·Journal of Neurology·Massimo Pandolfo
Mar 4, 2011·Movement Disorders : Official Journal of the Movement Disorder Society·Giovanni RizzoRaffaele Lodi
May 10, 2011·Brain Research Reviews·Daniele Marmolino
Jun 4, 2011·Acta Neuropathologica·Arnulf H KoeppenJennifer A Morral
Jan 22, 2013·Journal of Neuropathology and Experimental Neurology·Arnulf H Koeppen, Joseph E Mazurkiewicz
May 4, 2013·Neurological Sciences : Official Journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology·Christian Clemm von HohenbergKarl Egger
Apr 23, 2014·Human Gene Therapy·Marguerite V Evans-GaleaMartin B Delatycki
May 2, 2014·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·C Bonilha da SilvaM C França
Mar 25, 2015·Parkinsonism & Related Disorders·Simone Carreiro Vieira KarutaHelio Afonso Ghizoni Teive

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