Characteristic magnetic resonance imaging findings in Machado-Joseph disease

Archives of Neurology
Y MurataS Nakamura

Abstract

To clarify the characteristic magnetic resonance imaging (MRI) findings in patients with Machado-Joseph disease (MJD) diagnosed by genetic analysis. Using MRI, we examined 31 patients genetically diagnosed as having MJD, 20 patients with sporadic olivopontocerebellar atrophy, and 26 control subjects. The MRIs of patients with MJD disclosed remarkably reduced width of the superior cerebellar peduncles, atrophy in the frontal and temporal lobes, diminished transverse diameter of the globus pallidus, and decreased anteroposterior and transverse diameters of the pons, which correlated with the width of the middle cerebellar peduncle. The width of the superior cerebellar peduncles also correlated with the diameter of the dentate or red nucleus in patients with MJD, but not in controls or in patients with sporadic olivopontocerebellar atrophy. On T2- and/or proton-weighted axial MR imaging, a high signal intensity in the transverse pontine fibers was observed in 14 (45.2%) of 31 patients with MJD and in all patients with sporadic olivopontocerebellar atrophy, but not in any controls. Affected afferent and efferent cerebellar tracts and atrophy of the frontal and temporal lobes and globus pallidus are characteristics of MRI of patient...Continue Reading

Citations

Apr 23, 2013·The Canadian Journal of Neurological Sciences. Le Journal Canadien Des Sciences Neurologiques·Pettarusp M WadiaAnthony E Lang
Jun 4, 2011·Orphanet Journal of Rare Diseases·Conceição Bettencourt, Manuela Lima
Feb 1, 2003·Survey of Ophthalmology·Michael S Vaphiades
Feb 24, 2001·Neuropathology : Official Journal of the Japanese Society of Neuropathology·M YamadaH Takahashi
Jan 22, 2003·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·D Lo CocoV La Bella
Feb 18, 2016·The Journal of Cell Biology·Carlos A MatosAna Luísa Carvalho
Jun 8, 2001·Movement Disorders : Official Journal of the Movement Disorder Society·K BürkJ Dichgans
Jun 18, 2011·Psychiatry Research·Carsten LukasHorst K Hahn
May 24, 2011·Parkinsonism & Related Disorders·Gaurav GoelSanjeev Jain
Oct 9, 2009·Parkinsonism & Related Disorders·Anelyssa D'AbreuIscia Lopes-Cendes
Feb 21, 2013·Brain : a Journal of Neurology·Kathrin ReetzUNKNOWN axia Study Group Investigators
Dec 9, 2008·Parkinsonism & Related Disorders·Oraporn Sitburana, William G Ondo
Sep 1, 2005·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·A InagakiH Inagaki
Nov 22, 2008·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Kenshi TerajimaTsutomu Nakada
Feb 4, 2009·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·Y-C LeeB-W Soong
May 17, 2008·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Anelyssa D'AbreuFernando Cendes
Jun 14, 2012·Movement Disorders : Official Journal of the Movement Disorder Society·Yoshitsugu OgawaSatoshi Kuwabara
Apr 11, 2012·Movement Disorders : Official Journal of the Movement Disorder Society·Luke A MasseyHans R Jäger
Jun 28, 2011·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Anelyssa D'AbreuFernando Cendes
Jun 6, 2013·Movement Disorders : Official Journal of the Movement Disorder Society·Sandra RoeskeMartina Minnerop
Sep 8, 2004·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Eduardo S GhisolfiDiogo R Lara
Jul 21, 2004·Parkinsonism & Related Disorders·Eun Ah LeeWon Yong Lee
Jul 21, 2004·Parkinsonism & Related Disorders·Martin Viau, Yvan Boulanger
Dec 3, 2011·Progress in Neurobiology·Maria do Carmo Costa, Henry L Paulson
Feb 15, 2002·Brain : a Journal of Neurology·Peter SchwenkreisLudger Schöls
Feb 13, 2014·Sleep Medicine·Diogo Fernandes dos SantosGilmar Fernandes do Prado
Apr 28, 2017·PloS One·Carmen Rodríguez-CuetoJavier Fernández-Ruiz
Jun 1, 2011·Arquivos de neuro-psiquiatria·Sarah Teixeira CamargosAntonio Carlos dos Santos
Jan 14, 2010·Arquivos de neuro-psiquiatria·Hélio A G Teive
Sep 5, 2001·Neurologic Clinics·A Colcher, T Simuni
Sep 8, 2017·The International Journal of Neuroscience·Flora Giocondo, Giuseppe Curcio
Oct 3, 2002·Movement Disorders : Official Journal of the Movement Disorder Society·Tricia M ZawackiLewis Sudarsky
Nov 21, 2007·Internal Medicine·Masaki TakaoBan Mihara
Apr 18, 2008·The Cerebellum·Susanne DöhlingerJörg B Schulz
May 29, 2008·Journal of Neurology·Carsten LukasLudger Schöls

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