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

Data collected through routine syndromic surveillance for influenza-like illness in the Micronesian United States-affiliated Pacific Islands highlighted out-of-season influenza outbreaks in the spring of 2019. This report describes the data collected through the World Health Organization’s Pacific Syndromic Surveillance System for the Commonwealth of the Northern Mariana Islands (CNMI), Guam, the Federated States of Micronesia (FSM) and the Republic of the Marshall Islands (RMI). Compared with historical data, more cases of influenza-like illness were observed in all four islands described here, with the highest number reported in Guam in week 9, CNMI and FSM in week 15, and RMI in week 19. The outbreaks predominantly affected those aged <20 years, with evidence from CNMI and RMI suggesting higher attack rates among those who were unvaccinated. Cases confirmed by laboratory testing suggested that influenza B was predominant, with 83% (99/120) of subtyped specimens classified as influenza B/Victoria during January–May 2019. These outbreaks occurred after the usual influenza season and were consistent with transmission patterns in Eastern Asia rather than those in Oceania or the United States of America, the areas typically associated with the United States-affiliated Pacific Islands due to their geographical proximity to Oceania and political affiliation with the United States of America. A plausible epidemiological route of introduction may be the high levels of international tourism from Eastern Asian countries recorded during these periods of increased influenza B/Victoria circulation. This report demonstrates the value of year-round surveillance for communicable diseases and underscores the importance of seasonal influenza vaccination, particularly among younger age groups.