3型脊髓小脑共济失调患者骨密度评估
Assessment of Bone Mineral Density of Patients with Spinocerebellar Ataxia Type 3.
作者信息
Farias Aline Ms, Appenzeller Simone, França Marcondes C, Martinez Alberto Rm, Etchebehere Elba E, Souza Thiago F, Santos Allan O
机构信息
Nuclear Medicine Division, Department of Radiology, University of Campinas (UNICAMP), Campinas, São Paulo, Brazil.
Rheumatology Division, Department of Internal Medicine, University of Campinas (UNICAMP), Campinas, São Paulo, Brazil
出版信息
J Mov Disord. 2019 Jan;12(1):43-46. doi: 10.14802/jmd.18041. Epub 2019 Jan 30.
OBJECTIVE
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.
METHODS
Clinical data, lumbar X-rays and BMD data were obtained in 30 patients with MJD.
RESULTS
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.
CONCLUSION
Low BMD and fractures are frequent among MJD patients, and careful management of BMD may be beneficial for these patients.
目的
马查多-约瑟夫病（MJD）是一种脊髓小脑共济失调症，而骨质疏松症是一种多因素疾病，可能会影响神经系统疾病患者。然而，尚未对MJD患者中骨质疏松症的发生率进行研究。本研究的目的是评估骨密度（BMD），并确定MJD患者临床因素与椎体骨折发生率之间的相关性。
方法
收集了30例MJD患者的临床数据、腰椎X线片和BMD数据。
结果
根据Z值，10例患者（33.3%）在至少一个研究部位显示骨密度低。Z值与体重指数直接相关，股骨颈Z值与胞嘧啶-腺嘌呤-鸟嘌呤（CAG）重复序列长度呈负相关。骨密度与其他临床因素之间无相关性。43%的患者报告有既往病理性骨折。5例患者（16.7%）经腰椎X线检查发现至少有一处骨折。
结论
MJD患者中骨密度低和骨折很常见，对骨密度进行仔细管理可能对这些患者有益。
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