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Elba E Etchebehere 1 Article
Assessment of Bone Mineral Density of Patients with Spinocerebellar Ataxia Type 3
Aline MS Farias, Simone Appenzeller, Marcondes C Fran├ža, Alberto RM Martinez, Elba E Etchebehere, Thiago F Souza, Allan O Santos
J Mov Disord. 2019;12(1):43-46.   Published online January 30, 2019
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AbstractAbstract PDF
Machado-Joseph disease (MJD) is a spinocerebellar ataxia, and osteoporosis is a multifactor disease that may affect patients with neurologic conditions. The frequency of osteoporosis among MJD patients, however, has not been studied. The purpose of this study is to evaluate bone mineral density (BMD) and identify correlations between clinical factors and frequency of vertebral fractures in patients with MJD.
Clinical data, lumbar X-rays and BMD data were obtained in 30 patients with MJD.
Ten patients (33.3%) showed low BMD in at least one of the sites studied based on Z-scores. The Z-score correlated directly with body mass index, and the femoral neck Z-score was inversely correlated with cytosine-adenine-guanine (CAG) expansion. There was no correlation between BMD and other clinical factors. Forty-three percent of the patients reported previous pathologic fractures. Five patients (16.7%) had at least one fracture detected by lumbar X-ray.
Low BMD and fractures are frequent among MJD patients, and careful management of BMD may be beneficial for these patients.


Citations to this article as recorded by  
  • Overview of the Clinical Approach to Individuals With Cerebellar Ataxia and Neuropathy
    Leslie J. Roberts, Michael McVeigh, Linda Seiderer, Ian H. Harding, Louise A. Corben, Martin Delatycki, David J. Szmulewicz
    Neurology Genetics.2022;[Epub]     CrossRef
  • Effects of Neurological Disorders on Bone Health
    Ryan R. Kelly, Sara J. Sidles, Amanda C. LaRue
    Frontiers in Psychology.2020;[Epub]     CrossRef

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