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Regional Brain and Spinal Cord Volume Loss in Spinocerebellar Ataxia Type 3.
Faber, Jennifer; Schaprian, Tamara; Berkan, Koyak; Reetz, Kathrin; França, Marcondes Cavalcante; de Rezende, Thiago Junqueira Ribeiro; Hong, Jiang; Liao, Weihua; van de Warrenburg, Bart; van Gaalen, Judith; Durr, Alexandra; Mochel, Fanny; Giunti, Paola; Garcia-Moreno, Hector; Schoels, Ludger; Hengel, Holger; Synofzik, Matthis; Bender, Benjamin; Oz, Gulin; Joers, James; de Vries, Jereon J; Kang, Jun-Suk; Timmann-Braun, Dagmar; Jacobi, Heike; Infante, Jon; Joules, Richard; Romanzetti, Sandro; Diedrichsen, Jorn; Schmid, Matthias; Wolz, Robin; Klockgether, Thomas.
Afiliación
  • Faber J; DZNE, German Center for Neurodegenerative Diseases, Bonn, Germany.
  • Schaprian T; Department of Neurology, University Hospital Bonn, Bonn, Germany.
  • Berkan K; DZNE, German Center for Neurodegenerative Diseases, Bonn, Germany.
  • Reetz K; DZNE, German Center for Neurodegenerative Diseases, Bonn, Germany.
  • França MC; Department of Neurology, RWTH Aachen University, Bonn, Germany.
  • de Rezende TJR; JARA-Brain Institute Molecular Neuroscience and Neuroimaging, Forschungszentrum Jülich, Jülich, Germany.
  • Hong J; Brazilian Institute of Neuroscience and Neurotechnology (BRAINN), Campinas, Brazil.
  • Liao W; Department of Neurology, University of Campinas, Campinas, Brazil.
  • van de Warrenburg B; Brazilian Institute of Neuroscience and Neurotechnology (BRAINN), Campinas, Brazil.
  • van Gaalen J; Department of Neurology, University of Campinas, Campinas, Brazil.
  • Durr A; Department of Neurology, Xiangya Hospital, Central South University, Changsha, China.
  • Mochel F; Department of Radiology, Xiangya Hospital, Central South University, Changsha, People's Republic of China.
  • Giunti P; Department of Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands.
  • Garcia-Moreno H; Department of Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition and Behaviour, Nijmegen, The Netherlands.
  • Schoels L; Sorbonne Université, Paris Brain Institute, AP-HP, INSERM, CNRS, Pitié-Salpêtrière University Hospital, Paris, France.
  • Hengel H; Sorbonne Université, Paris Brain Institute, AP-HP, INSERM, CNRS, Pitié-Salpêtrière University Hospital, Paris, France.
  • Synofzik M; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
  • Bender B; National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London, United Kingdom.
  • Oz G; Ataxia Centre, Department of Clinical and Movement Neurosciences, UCL Queen Square Institute of Neurology, London, United Kingdom.
  • Joers J; National Hospital for Neurology and Neurosurgery, University College London Hospitals NHS Foundation Trust, London, United Kingdom.
  • de Vries JJ; Department of Neurodegenerative Diseases and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
  • Kang JS; German Centre for Neurodegenerative Diseases (DZNE), Tübingen, Germany.
  • Timmann-Braun D; Department of Neurodegenerative Diseases and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
  • Jacobi H; German Centre for Neurodegenerative Diseases (DZNE), Tübingen, Germany.
  • Infante J; Department of Neurodegenerative Diseases and Hertie-Institute for Clinical Brain Research, University of Tübingen, Tübingen, Germany.
  • Joules R; German Centre for Neurodegenerative Diseases (DZNE), Tübingen, Germany.
  • Romanzetti S; Department of Diagnostic and Interventional Neuroradiology, University Hospital Tübingen, Tübingen, Germany.
  • Diedrichsen J; Center for Magnetic Resonance Research, Department of Radiology, University of Minnesota, Minneapolis, Minnesota, USA.
  • Schmid M; Center for Magnetic Resonance Research, Department of Radiology, University of Minnesota, Minneapolis, Minnesota, USA.
  • Wolz R; Department of Neurology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
  • Klockgether T; Department of Neurology, Goethe University, Frankfurt am Main, Germany.
Mov Disord ; 36(10): 2273-2281, 2021 10.
Article en En | MEDLINE | ID: mdl-33951232
BACKGROUND: Given that new therapeutic options for spinocerebellar ataxias are on the horizon, there is a need for markers that reflect disease-related alterations, in particular, in the preataxic stage, in which clinical scales are lacking sensitivity. OBJECTIVE: The objective of this study was to quantify regional brain volumes and upper cervical spinal cord areas in spinocerebellar ataxia type 3 in vivo across the entire time course of the disease. METHODS: We applied a brain segmentation approach that included a lobular subsegmentation of the cerebellum to magnetic resonance images of 210 ataxic and 48 preataxic spinocerebellar ataxia type 3 mutation carriers and 63 healthy controls. In addition, cervical cord cross-sectional areas were determined at 2 levels. RESULTS: The metrics of cervical spinal cord segments C3 and C2, medulla oblongata, pons, and pallidum, and the cerebellar anterior lobe were reduced in preataxic mutation carriers compared with controls. Those of cervical spinal cord segments C2 and C3, medulla oblongata, pons, midbrain, cerebellar lobules crus II and X, cerebellar white matter, and pallidum were reduced in ataxic compared with nonataxic carriers. Of all metrics studied, pontine volume showed the steepest decline across the disease course. It covaried with ataxia severity, CAG repeat length, and age. The multivariate model derived from this analysis explained 46.33% of the variance of pontine volume. CONCLUSION: Regional brain and spinal cord tissue loss in spinocerebellar ataxia type 3 starts before ataxia onset. Pontine volume appears to be the most promising imaging biomarker candidate for interventional trials that aim at slowing the progression of spinocerebellar ataxia type 3. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Machado-Joseph / Ataxias Espinocerebelosas Límite: Humans Idioma: En Revista: Mov Disord Asunto de la revista: NEUROLOGIA Año: 2021 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Estados Unidos
Texto completo
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad de Machado-Joseph / Ataxias Espinocerebelosas Límite: Humans Idioma: En Revista: Mov Disord Asunto de la revista: NEUROLOGIA Año: 2021 Tipo del documento: Article País de afiliación: Alemania Pais de publicación: Estados Unidos