Feleus Stephanie, van Schaijk Malu, Roos Raymund A C, de Bot Susanne T
Department of Neurology, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, The Netherlands.
Department of Epidemiology, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, The Netherlands.
J Pers Med. 2022 Apr 6;12(4):589. doi: 10.3390/jpm12040589.
Huntington's Disease (HD) is a rare, neurodegenerative disorder characterized by chorea, cognitive decline, and behavioral changes. Despite wide clinical use since the mid-1980s, tiapride was recently withdrawn from the Dutch market without rationale. Although alternatives are available, many patients experienced dysregulation after this unwanted change. We provide insight into the impact of sudden tiapride withdrawal by reviewing medical records of HD patients who were using tiapride at the time of withdrawal. In addition, we performed a systematic search in five databases on tiapride efficacy and its safety profile in HD. Original research and expert opinions were included. In our patient group on tiapride, 50% required tiapride import from abroad. Regarding the review, 12 articles on original datasets and three expert opinions were included. The majority of studies showed an improvement in chorea while patients were on tiapride. Due to limited sample sizes, not all studies performed statistical tests on their results. Fifty percent of clinical experts prefer tiapride as initial chorea monotherapy, especially when comorbid behavioral symptoms are present. Side effects are often rare and mild. No safety concerns were reported. In conclusion, tiapride is almost irreplaceable for some patients and is an effective and safe chorea treatment in HD.
亨廷顿舞蹈症(HD)是一种罕见的神经退行性疾病,其特征为舞蹈症、认知衰退和行为改变。自20世纪80年代中期以来,舒必利在临床上广泛使用,但最近在荷兰市场上被无故撤市。尽管有其他替代药物,但许多患者在这一意外变化后出现了失调症状。我们通过回顾撤市时正在使用舒必利的HD患者的病历,深入了解了突然停用舒必利的影响。此外,我们在五个数据库中对舒必利在HD中的疗效及其安全性进行了系统检索。纳入了原始研究和专家意见。在我们使用舒必利的患者组中,50%需要从国外进口舒必利。关于此次综述,纳入了12篇关于原始数据集的文章和三篇专家意见。大多数研究表明,患者在使用舒必利时舞蹈症有所改善。由于样本量有限,并非所有研究都对其结果进行了统计检验。50%的临床专家更喜欢将舒必利作为舞蹈症的初始单一疗法,尤其是当存在合并行为症状时。副作用通常很少且轻微。未报告安全问题。总之,舒必利对一些患者来说几乎是不可替代的,并且是治疗HD舞蹈症的一种有效且安全的药物。