J Mov Disord.  2019 Jan;12(1):43-46. 10.14802/jmd.18041.

Assessment of Bone Mineral Density of Patients with Spinocerebellar Ataxia Type 3

• Farias AM
• Appenzeller S
• França MC
• Martinez A
• Etchebehere EE
• Souza TF
• Santos AO

• KMID: 2438678
• DOI: http://doi.org/10.14802/jmd.18041

Abstract

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.

Keyword

Machado-Joseph disease; osteoporosis; spinocerebellar ataxia type 3

MeSH Terms

Body Mass Index
Bone Density*
Femur Neck
Fractures, Spontaneous
Humans
Machado-Joseph Disease*
Osteoporosis
Spinocerebellar Ataxias*