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抗 N-甲基-D-天冬氨酸受体脑炎中运动障碍的变化:台湾地区的一项全国性研究。

Variations of movement disorders in anti-N-methyl-D-aspartate receptor encephalitis: A nationwide study in Taiwan.

作者信息

Duan Bi-Chun, Weng Wen-Chin, Lin Kuang-Lin, Wong Lee Chin, Li Sung-Tse, Hsu Mei-Hsin, Lin Jainn-Jim, Fan Pi-Chaun, Lin Ming-I, Chiu Nan-Chang, Lin Yu-Ching, Wang Huei-Shyong, Hung Kun-Long, Lee Wang-Tso

机构信息

Department of Pediatric Neurology, National Taiwan University Children's Hospital, Taipei Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taoyuan Department of Pediatrics, Hsinchu Mackay Memorial Hospital, Hsinchu Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung Department of Pediatrics, Shin Kong Wu Ho-Su Memorial Hospital, Taipei, Taiwan Department of Pediatrics, Mackay Memorial Hospital, Taipei Department of Physical Medicine and Rehabilitation, National Cheng Kung University Hospital, College of Medicine, National Cheng Kung University, Tainan Department of Pediatrics, Cathay General Hospital, Taipei, Taiwan.

出版信息

Medicine (Baltimore). 2016 Sep;95(37):e4365. doi: 10.1097/MD.0000000000004365.

Abstract

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitis that presents with a wide variety of movement disorders. The purpose of our study is to review the manifestations and duration of movement disorders in different ages with NMDAR encephalitis.A retrospective cohort of 28 patients (20 females and 8 males) with positive cerebrospinal fluid (CSF) anti-NMDAR antibody in a 5-year period from major hospitals in Taiwan was enrolled. They were categorized into 3 age groups: 7 patients were ≤10 years, 14 patients were 10 to 18 years, and 7 patients were >18 years.Total 28 patients (20 females and 8 males) with age ranging from 8 months to 38 years were enrolled. Nearly all patients (n = 27/28, 96%) presented with at least 2 types of disorders, including orofacial-lingual dyskinesia (OFLD; n = 20), catatonia (n = 19), tremor (n = 11), bradykinesia (n = 11), dystonia (n = 11), choreoathethosis (n = 9), and ballism (n = 3). Only 1 patient below 10 years presented with isolated periodic choreoathethosis without other movement disorders. OFLD was common in all age groups. Choreoathetosis was most common in patients aged ≤10 years, while catatonia was most common in patients aged >10 years (P = 0.001 and 0.020, respectively). Bradykinesia was also more common in patients aged >10 years (P = 0.020). The clinical presentations of movement disorders were not significantly different in the age of 10 to 18 years and those >18 years. Neither patient ≤10 years old nor male patients had associated tumors. All patients' movement disorders were improved after treatment, while female patients with tumors had worse short-term outcome (P = 0.014). Compared with other disorders, choreoathetosis persisted significantly longer in patients ≤10 years (P = 0.038), while OFLD and catatonia last longer in patients >10 years (P = 0.047 and 0.002, respectively).Our study shows that hyperkinetic movements such as choreoathetosis are more common and last longer in younger age groups, whereas hypokinetic movements such as catatonia and bradykinesia are more common and last longer in older age groups. Female patients with ovarian tumors had worse short-term outcome.

摘要

抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎是最常见的自身免疫性脑炎之一,可出现多种运动障碍。我们研究的目的是回顾不同年龄NMDAR脑炎患者运动障碍的表现及持续时间。

纳入台湾各大医院5年期间脑脊液(CSF)抗NMDAR抗体阳性的28例患者(20例女性,8例男性)。他们被分为3个年龄组:7例年龄≤10岁,14例年龄为10至18岁,7例年龄>18岁。

共纳入28例年龄从8个月至38岁的患者(20例女性,8例男性)。几乎所有患者(n = 27/28,96%)至少出现2种类型的障碍,包括口面部-舌运动障碍(OFLD;n = 20)、紧张症(n = 19)、震颤(n = 11)、运动迟缓(n = 11)、肌张力障碍(n = 11)、舞蹈手足徐动症(n = 9)和投掷症(n = 3)。仅1例10岁以下患者表现为孤立的周期性舞蹈手足徐动症,无其他运动障碍。OFLD在所有年龄组中都很常见。舞蹈手足徐动症在年龄≤10岁的患者中最常见,而紧张症在年龄>10岁的患者中最常见(分别为P = 0.001和0.020)。运动迟缓在年龄>10岁的患者中也更常见(P = 0.020)。10至18岁年龄组与>18岁年龄组的运动障碍临床表现无显著差异。≤10岁的患者和男性患者均无相关肿瘤。所有患者的运动障碍经治疗后均有改善,而患有肿瘤的女性患者短期预后较差(P = 0.014)。与其他障碍相比,舞蹈手足徐动症在≤10岁的患者中持续时间显著更长(P = 0.038),而OFLD和紧张症在>10岁的患者中持续时间更长(分别为P = 0.047和0.002)。

我们的研究表明,舞蹈手足徐动症等运动过多性运动在较年轻年龄组中更常见且持续时间更长,而紧张症和运动迟缓等运动过少性运动在较年长年龄组中更常见且持续时间更长。患有卵巢肿瘤的女性患者短期预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c855/5402545/0023d90b0b8f/medi-95-e4365-g002.jpg

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