Multicenter questionnaire survey for sporadic inclusion body myositis in Japan

Background: Sporadic inclusion body myositis (sIBM) is the most prevalent acquired muscle disease in the elderly. sIBM is an intractable and progressive disease of unknown cause and without effective treatment. The etiology of sIBM is still unknown; however, genetic factors, aging, lifestyles, and environmental factors may be involved. The purpose of this study is to elucidate the cross-sectional profile of patients affected by sIBM in Japan. Methods: We surveyed patient data for 146 cases diagnosed at a number of centers across Japan. We also issued a questionnaire for 67 patients and direct caregivers to further elucidate the natural history of the disease. Results: The mean age at the onset was 63.4 ± 9.2 years. The mean length of time from the onset to diagnosis was 55.52 ± 49.72 months, suggesting that there is a difficulty in diagnosing this disease with long-term consequences because of late treatment. 73 % described the psychological/mental aspect of the disease. The most popular primary caregiver was the patient’s spouse and 57 % patients mentioned that they were having problems managing the finances. Conclusions: Through these surveys, we described the cross-sectional profiles of sIBM in Japan. Many patients described psychological/mental and financial anxiety because of the aged profile of sIBM patients. The profiles of sIBM patients are similar to those in Western countries

The updated retrospective questionnaire study of sporadic inclusion body myositis in Japan

Background: Sporadic inclusion body myositis (sIBM) is the most prevalent muscle disease in elderly people, affecting the daily activities. sIBM is progressive with unknown cause and without effective treatment. In 2015, sIBM was classified as an intractable disease by the Japanese government, and the treatment cost was partly covered by the government. This study aimed to examine the changes in the number of patients with sIBM over the last 10 years and to elucidate the cross-sectional profile of Japanese patients with sIBM. Methods: The number of sIBM patients was estimated through a reply-paid postcard questionnaire for attending physicians. Only patients diagnosed as “definite” or “probable” sIBM by clinical and biopsy sIBM criteria were included in this study (Lancet Neurol 6:620-631, 2007, Neuromuscul Disord 23:1044-1055, 2013). Additionally, a registered selfadministered questionnaire was also sent to 106 patients who agreed to reply via their attending physician, between November 2016 and March 2017. Results: The number of patients diagnosed with sIBM for each 5-year period was 286 and 384 in 2011 and 2016, respectively. Inability to stand-up, cane-dependent gait, inability to open a plastic bottle, choking on food ingestion, and being wheelchair-bound should be included as sIBM milestones. Eight patients were positive for anti-hepatitis C virus antibody; three of them were administered interferon before sIBM onset. Steroids were administered to 33 patients (31.1%) and intravenous immunoglobulin to 46 patients (43.4%). From 2016 to 2017, total of 70 patients applied for the designated incurable disease medical expenses subsidy program. Although the treatment cost was partly covered by the government, many patients expressed psychological/mental and financial anxieties. Conclusions: We determined the cross-sectional profile of Japanese patients with sIBM. Continuous support and prospective surveys are warranted

Motor planning error in Parkinson's disease and its clinical correlates.

This study aimed to investigate (a) motor planning difficulty by using a two-step test in Parkinson's disease (PD) compared with age-matched healthy subjects and (b) the relationship between motor planning difficulty and clinical factors. The two-step test was performed by 58 patients with PD with Hoehn & Yahr (H&Y) stage I-IV and 110 age-matched healthy older adult controls. In the two-step test, the participants estimated the two-step distance with maximum effort. Subsequently, they performed the actual two-step trial to measure the actual maximum distance. We calculated the accuracy of the estimation (estimated distance minus actual distance). In both groups, subjects who estimated >5 cm were defined as the overestimation group, and those who estimated <5 cm over- and underestimation were defined as the non-overestimation group. The overestimation group consisted of 17 healthy older adults (15.5%) and 23 patients with PD (39.7%). The number of patients with PD with overestimation was significantly more than that of healthy controls by Chi-squared test. H&Y stage and the Unified Parkinson's Disease Rating Scale (UPDRS) part II and III scores in overestimation group in PD patients were significantly higher than those in overestimation group in PD patients. Moreover, multiple regression using H&Y stage and UPDRS parts II and III as independent variables showed that the UPDRS part II score was the only related factor for the estimation error distance. Estimation error distance was significant correlated with UPDRS parts II and III. Patients with PD easily have higher rates of motor-related overestimation than age-matched healthy controls. In addition, UPDRS parts II and III expressed ability of activities of daily living and motor function as influences on motor-related overestimation. Particularly, multiple regression indicated that UPDRS part II directly showed the ability of daily living as an essential factor for overestimation