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https://www.selleckchem.com/products/eidd-2801.html Both exposure to low (RR 2.045, 95% CI 1.690, 2.474) and high temperatures (RR 1.244, 95% CI 1.107, 1.399) were associated with increased risk of total inflammation-related visits in Haiyuan county. Low temperatures were associated with increased risks of all types of inflammation-related diseases in Yanchi county (RR 4.344, 95% CI 2.887, 6.535), while high temperatures only affected gastroenteritis (RR 1.274, 95% CI 1.040, 1.561). Moderate temperatures explained approximately 26% and 33% of clinical visits due to inflammation-related diseases in Haiyuan and Yanchi, respectively, with the burden attributable to cold exposure higher than hot exposure. The reference temperature values ranged from 17 to 19 in Haiyuan, and 12 to 14 in Yanchi for all types of clinical visits. CONCLUSIONS Our findings add additional evidence for the adverse effect of suboptimal ambient temperature and provide useful information for public health programs targeting people living in rural villages. INTRODUCTION In 2011, a homozygous mutation in GOSR2 (c.430G > T; p. Gly144Trp) was reported as a novel cause of Progressive Myoclonus Epilepsy (PME) with early-onset ataxia. Interestingly, the ancestors of patients originate from countries bound to the North Sea, hence the condition was termed North Sea PME (NSPME). Until now, only 20 patients have been reported in literature. Here, we provide a detailed description of clinical and neurophysiological data of seventeen patients. METHODS We collected clinical and neurophysiological data from the medical records of seventeen NSPME patients (5-46 years). In addition, we conducted an interview focused on factors influencing myoclonus severity. RESULTS The core clinical features of NSPME are early-onset ataxia, myoclonus and seizures, with additionally areflexia and scoliosis. Factors such as fever, illness, heat, emotions, stress, noise and light (flashes) all exacerbated myoclonic jerks. Epilepsy s

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