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Muscular dystrophy (MD) is a genetic disorder that causes progressive muscle weakness and degeneration. Limb‐girdle muscular dystrophy (LGMD) is a type of MD that mainly causes muscle atrophy within the shoulder and pelvic girdles. LGMD is classified into autosomal dominant (LGMD‐D) and autosomal recessive (LGMD‐R) inheritance patterns. Mutations in the Dysferlin gene (DYSF) are common causes of LGMD‐R. However, genetic screening of DYSF mutations is rare in Taiwan. Herein, we identified a novel c.2867_2871del ACCAG deletion and a previously reported c.937+1G>A mutation in DYSF from a Taiwanese family with LGMD. The primary symptoms of both siblings were difficulty climbing stairs, walking on the toes, and gradually worsening weakness in the proximal muscles and increased creatine kinase level. Through pedigree analysis and sequencing, two siblings from this family were found to have compound heterozygous DYSF mutations (c. 937+1G>A and c. 2867_2871del ACCAG) within the separated alleles. These mutations induced early stop codons; if translated, truncated DYSF proteins will be expressed. Or, the mRNA products of these two mutations will merit the nonsense‐mediated decay, might result in no dysferlin protein expressed. To our knowledge, this is the first report of a novel c.2867_2871del ACCAG deletion in DYSF. Further research is required to examine the effects of the novel DYSF mutation in Taiwanese patients with LGMD. [ABSTRACT FROM AUTHOR]

Background: Coffee and tea consumption has been shown to associate with the risk of dementia. However, little is known about how sex and vascular comorbidities (type 2 diabetes, hypertension, and hypercholesterolemia) modify this association. Method: This is a multi‐center case‐control study. We recruited 278 Alzheimer's disease (AD) and 102 small‐vessel vascular dementia (VaD) patients aged 60 or older from three teaching hospitals in Taiwan from 2007 to 2010; controls (n = 468) were recruited from the health checkup program during the same period of time. The outcomes of this study are clinically diagnosed AD and VaD. Information on coffee and tea consumption and the history of type 2 diabetes, hypertension, and hypercholesterolemia were collected based on questionnaires and medical charts. A multinomial logistic regression model was utilized to evaluate 1) the association of coffee and tea consumption with dementia risk, and 2) how sex and vascular risk factors modified these associations. Result: Different combinations and quantities of coffee and tea consumption protected against AD and VaD. Our analyses demonstrated consumption of three or more cups of coffee or tea per day was associated with a decreased risk of AD [adjusted odds ratio (aOR) = 0.42; 95% confidence interval (CI) = 0.22‐0.78)] and VaD (aOR = 0.42; 95% CI = 0.19‐0.94). After stratification, these associations became more evident in females for AD (aOR = 0.15; 95% CI = 0.05‐0.46) and in males for VaD (aOR = 0.06; 95% CI = 0.01‐0.50). Similarly, hypertensive participants consuming higher quantities of coffee and tea had a decreased risk of AD (aOR = 0.30; 95% CI = 0.11‐0.78). Moreover, hypercholesterolemia significantly modified the association of coffee or tea consumption with the risk of dementia (Pinteraction < 0.001). Conclusion: Coffee and tea consumption was associated with a decreased risk of AD and VaD. Sex and vascular comorbidities differentially modified the association of coffee and tea consumption with the risk of dementia. [ABSTRACT FROM AUTHOR]

Aim: To determine whether giving dementia caregivers active psychoeducational intervention is more efficacious than passive intervention for improving their caregiving skills and reducing their caregiving burden. Methods: This study was a prospective, single‐blinded, controlled trial with 43 caregiver/person‐with‐dementia dyads. The dyads were randomly assigned to the active psychoeducational intervention (AP) group, which used role‐play, discussion, and development of problem‐solving capacity to build up their caregiving skills and competence, or the passive psychoeducational intervention (PP) group, which gave caregivers educational materials on common caregiving strategies. Primary outcomes were the levels of caregiver competence (Care Skill Inventory [CSI]), burden (Chinese Zarit Burden Inventory [CZBI]), and distress caused by the behavioral and psychological symptoms of dementia (Neuropsychiatric Inventory‐Questionnaire [NPI‐Q]). Outcomes were assessed pre‐test, post‐test and after 3 months. Repeated measures one‐way analysis of variance was used to compare mean‐change scores between time‐points, and generalized estimating equations (GEE) were used to compare groups. Results: Post‐test or 3‐month (or both) Care Skill Inventory, Chinese Zarit Burden Inventory and Neuropsychiatric Inventory‐Questionnaire distress levels were significantly (p < 0.05) better in the AP but not in the PP group. The generalized estimating equation intergroup comparison, adjusted for potential confounders, showed that Care Skill Inventory in the AP group was more significantly improved than in the PP group, and that Chinese Zarit Burden Inventory nearly reached significance. Conclusions: Active rather than passive psychoeducation, even in a short (3 months) intervention of six visits, was more efficacious for improving caregiving competence. Future studies will require larger samples. Geriatr Gerontol Int 2018; 18: 750–757. [ABSTRACT FROM AUTHOR]

Background Increasing evidence shows that bilingualism or multilingualism may have beneficial effects on preventing dementia. We performed a cross-sectional, community-based study in Taiwan. Some elders (older than 70 years) in Taiwan can speak Japanese because of the formal Japanese education they received before World War II, when Taiwan was under Japanese rule. After the war, Mandarin Chinese was adopted as the official language of Taiwan. We assessed whether constantly using three languages had an effect on dementia prevalence and cognitive function. Methods We defined multilingualism as the ability to fluently speak Taiwanese (T), Japanese (J), and Mandarin Chinese (C) in daily life. We evaluated the Mini-Mental State Examination and AD8 questionnaire results of 514 community-dwelling people older than 70 years in Taishan, Taiwan. Results Seventy-three of the subjects (14.2%) were multilingual (T, J, C) and 441 (85.8%) were bilingual (T, C). No difference was noted in dementia prevalence between multilingual (6.8%) and bilingual (7.4%) populations, but multilinguals were older than bilinguals (mean age: 79.9 vs 77.3 years). Multilinguals had higher Mini-Mental State Examination scores than bilinguals (mean: 24.6 vs. 22.7). However, after the subjects were stratified into low and high education level groups, the Mini-Mental State Examination difference was found to be significant in only the low education level group. Conclusions Dementia prevalence did not significantly differ between the multilingual (T, J, C) and bilingual (T, C) groups. However, given that the average age of the multilingual group was approximately 2 years older than that of the bilingual group, there may have been minor effects in the multilingual group. [ABSTRACT FROM AUTHOR]

Stenting is a potential alternative treatment for carotid artery stenosis. Direct stenting may, theoretically, reduce the risk of embolism by minimizing plaque manipulation before tissue scaffolding is achieved. The results of direct carotid stenting are reported and compared with those of stenting with predilatation. One hundred and seventy-four carotid artery stenoses were treated from July 1998 to February 2002, with 84 lesions directly stented (Group 1) and the other 90 lesions stented after predilatation (Group 2). The criteria for direct stenting were minimal luminal diameter (MLD) > 1 mm and no visible thrombus angiographically. Technical success rates of the two groups were both 100%, without any cross-over. Reference vessel diameter and lesion length did not differ between the two groups. In Group 1, diameter stenosis was lower (79 ± 8 vs 92 ± 7%,P < 0.001) and MLD was larger (1.1 ± 0.5 vs 0.4 ± 0.4 mm,P < 0.001) than that in Group 2, but the final MLD (4.7 ± 0.9 vs 4.7 ± 0.9 mm,P = 0.94) of the two groups were not statistically different. The periprocedural ipsilateral stroke or death rates were also similar in the two groups (2/84 vs 4/90,P = 0.68). It was concluded that if the MLD of carotid stenosis is larger than 1 mm and no thrombus is present, direct stenting could be carried out safely with results comparable to that of stenting after predilatation. [ABSTRACT FROM AUTHOR]

High-dose corticosteroid administration has been regarded as the main therapy for acute disseminated encephalomyelitis (ADEM). However, some patients with ADEM do not respond well to this treatment. We successfully used plasmapheresis to treat 2 patients who had ADEM. We also compared our patients' symptoms and clinical outcomes to those from previous reports. Plasmapheresis may be indicated not only for severe cases that fail to improve after high-dose corticosteroid treatment but also for first-line treatment. Additional large, controlled, double-blinded trials are needed to clarify the role of plasmapheresis in ADEM. J Clin. Apheresis 19:154-159, 2004. © 2004 Wiley-Liss, Inc. [ABSTRACT FROM AUTHOR]