PDF(Pubmed)
Abstract：
UNASSIGNED: The effect of stiff person syndrome spectrum disorders (SPSD) on the gastrointestinal tract (GIT) is unknown. This case series aims to characterize the prevalence and types of GI dysfunction in individuals with SPSD.
UNASSIGNED: A retrospective chart review included individuals diagnosed with SPSD with descriptors of GI symptoms in their medical records. SPSD phenotypes, type of motility test performed, and dysmotility pattern (upper, lower, or diffuse) were assessed. Descriptive statistics and univariate chi-square analyses were utilized.
UNASSIGNED: Of 240 individuals with SPSD, 32% reported GI symptoms, most were female (83.1%), and white (74%), with a median age at time of GI symptom onset of 50 ± 13 years. Most common symptoms reported were dysphagia (45%), constipation (40%), and nausea/vomiting (23%). Most individuals had classic SPS (47%) followed by SPS-plus (29%) and 82.9% were positive for serum antiGAD65 antibodies. Of 36 patients that underwent at least one GI motility test, 26 had evidence of upper, lower, or diffuse GI dysmotility (44.4%, 17%, and 4%, respectively). The group who did not undergo testing had a higher proportion of patients with DM.
UNASSIGNED: There is a high prevalence of GI symptoms and transit abnormalities in patients with SPSD. Future prospective, longitudinal studies are warranted to further assess GI symptoms in the context of SPSD and to determine if individuals with GI symptoms differ in prognosis or treatment response from those without GI symptoms. In the meantime, there should be a low threshold for motility testing in patients with SPSD.
UNASSIGNED: A retrospective chart review included individuals diagnosed with SPSD with descriptors of GI symptoms in their medical records. SPSD phenotypes, type of motility test performed, and dysmotility pattern (upper, lower, or diffuse) were assessed. Descriptive statistics and univariate chi-square analyses were utilized.
UNASSIGNED: Of 240 individuals with SPSD, 32% reported GI symptoms, most were female (83.1%), and white (74%), with a median age at time of GI symptom onset of 50 ± 13 years. Most common symptoms reported were dysphagia (45%), constipation (40%), and nausea/vomiting (23%). Most individuals had classic SPS (47%) followed by SPS-plus (29%) and 82.9% were positive for serum antiGAD65 antibodies. Of 36 patients that underwent at least one GI motility test, 26 had evidence of upper, lower, or diffuse GI dysmotility (44.4%, 17%, and 4%, respectively). The group who did not undergo testing had a higher proportion of patients with DM.
UNASSIGNED: There is a high prevalence of GI symptoms and transit abnormalities in patients with SPSD. Future prospective, longitudinal studies are warranted to further assess GI symptoms in the context of SPSD and to determine if individuals with GI symptoms differ in prognosis or treatment response from those without GI symptoms. In the meantime, there should be a low threshold for motility testing in patients with SPSD.
摘要：
■僵硬的人综合征谱系障碍（SPSD）对胃肠道（GIT）的影响尚不清楚。本病例系列旨在描述SPSD患者胃肠道功能障碍的患病率和类型。
■回顾性图表回顾包括诊断为SPSD的个体，其医疗记录中有胃肠道症状的描述。SPSD表型,进行的运动性测试类型，和运动障碍模式(上部，较低,或弥漫性)进行评估。利用描述性统计和单变量卡方分析。
■在240名SPSD患者中，32%报告了胃肠道症状，大多数是女性(83.1%)，白人（74%）胃肠道症状发作时的中位年龄为50±13岁。报告的最常见症状是吞咽困难（45％），便秘（40%），恶心/呕吐(23%)。大多数个体有经典SPS（47％），其次是SPS-plus（29％）和82.9％的血清抗GAD65抗体阳性。在36名接受至少一次胃肠动力测试的患者中，26有上层的证据，较低,或弥漫性胃肠动力障碍(44.4%，17%,4%,分别)。未接受测试的组的DM患者比例较高。
■在SPSD患者中，胃肠道症状和转运异常的患病率很高。未来的前景，有必要进行纵向研究,以进一步评估SPSD背景下的胃肠道症状,并确定有胃肠道症状的个体与无胃肠道症状的个体在预后或治疗反应方面是否存在差异.同时,SPSD患者的运动试验阈值应该较低.
■回顾性图表回顾包括诊断为SPSD的个体，其医疗记录中有胃肠道症状的描述。SPSD表型,进行的运动性测试类型，和运动障碍模式(上部，较低,或弥漫性)进行评估。利用描述性统计和单变量卡方分析。
■在240名SPSD患者中，32%报告了胃肠道症状，大多数是女性(83.1%)，白人（74%）胃肠道症状发作时的中位年龄为50±13岁。报告的最常见症状是吞咽困难（45％），便秘（40%），恶心/呕吐(23%)。大多数个体有经典SPS（47％），其次是SPS-plus（29％）和82.9％的血清抗GAD65抗体阳性。在36名接受至少一次胃肠动力测试的患者中，26有上层的证据，较低,或弥漫性胃肠动力障碍(44.4%，17%,4%,分别)。未接受测试的组的DM患者比例较高。
■在SPSD患者中，胃肠道症状和转运异常的患病率很高。未来的前景，有必要进行纵向研究,以进一步评估SPSD背景下的胃肠道症状,并确定有胃肠道症状的个体与无胃肠道症状的个体在预后或治疗反应方面是否存在差异.同时,SPSD患者的运动试验阈值应该较低.
