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[一个走路步态怪异的腿部僵硬的男子]

[A stiff-legged man with a bizarre gait].

作者信息

Holmøy Trygve, Horn Morten Andreas, Vandvik Bodvar

机构信息

Nevrologisk avdeling, Ullevål universitetssykehus, 0407 Oslo.

出版信息

Tidsskr Nor Laegeforen. 2007 May 31;127(11):1529-30.

PMID:17566229
Abstract

BACKGROUND

Stiff person syndrome (SPS) is a rare neurological disorder characterized by simultaneous contraction of agonistic and antagonistic muscles. SPS can easily be confused with a psychogenic movement disorder, but the frequent finding of autoantibodies against glutamic acid decarboxylase (GAD65), positive response to immunomodulatory treatment and association with other autoimmune diseases strongly suggests an immununological pathogenesis.

CASE REPORT

A 43-year-old man was admitted with a three-year history of stiffness and painful spasms in the left leg, causing walking problems and frequent falls. A clinical examination revealed a bizarre gait, pes equinovarus, and simultaneous contraction of agonistic and antagonistic muscles, but no other neurological deficits. Electromyography was normal except for simultaneous contraction of agonistic and antagonistic muscles. Magnetic resonance imaging of the neuraxis and routine examinations of blood and cerebrospinal fluid were normal. A diagnosis of conversion neurosis was considered, but was not supported by positive evidence. The symptoms gradually evolved to affect the back and both legs. Elevated levels of antibodies against GAD65 in serum and cerebrospinal fluid confirmed the diagnosis SPS three and a half years after symptom debut. Both stiffness and muscle cramps responded excellently to diazepam.

CONCLUSION

This report calls attention to a rare neurological disease, in which absence of specific neurological deficits may lead to an erroneous diagnosis of a psychogenic disorder.

摘要

背景

僵人综合征(SPS)是一种罕见的神经系统疾病,其特征为拮抗肌和主动肌同时收缩。SPS很容易与精神性运动障碍相混淆,但谷氨酸脱羧酶65(GAD65)自身抗体的频繁检出、免疫调节治疗的阳性反应以及与其他自身免疫性疾病的关联,强烈提示其发病机制与免疫有关。

病例报告

一名43岁男性因左腿僵硬和疼痛性痉挛3年入院,导致行走困难和频繁跌倒。临床检查发现步态怪异、马蹄内翻足,拮抗肌和主动肌同时收缩,但无其他神经功能缺损。除拮抗肌和主动肌同时收缩外,肌电图检查正常。神经轴的磁共振成像以及血液和脑脊液的常规检查均正常。曾考虑诊断为转换性神经症,但无阳性证据支持。症状逐渐发展至累及背部和双腿。血清和脑脊液中抗GAD65抗体水平升高,在症状出现三年半后确诊为SPS。地西泮对僵硬和肌肉痉挛均有良好疗效。

结论

本报告提醒注意一种罕见的神经系统疾病,其中缺乏特异性神经功能缺损可能导致对精神性障碍的误诊。

相似文献

1
[A stiff-legged man with a bizarre gait].[一个走路步态怪异的腿部僵硬的男子]
Tidsskr Nor Laegeforen. 2007 May 31;127(11):1529-30.
2
The stiff-person syndrome. Case report.僵人综合征。病例报告。
Minerva Anestesiol. 2002 Nov;68(11):861-5.
3
['Stiff-person'-syndrome].“僵人综合征”
Ned Tijdschr Geneeskd. 2003 Jun 21;147(25):1228-32.
4
[Two stiff person cases misdiagnosed as conversion disorder].[两例被误诊为转换障碍的僵人综合征病例]
Turk Psikiyatri Derg. 2009 Winter;20(4):392-7.
5
Focal stiff-person syndrome.局灶性僵人综合征。
Neurologia. 2001 Feb;16(2):89-91.
6
Stiff person syndrome: avoiding misdiagnosis.僵人综合征:避免误诊
Neurol Sci. 2007 Mar;28(1):35-7. doi: 10.1007/s10072-007-0745-9.
7
[Stiff person syndrome--case report].[僵人综合征——病例报告]
Pol Merkur Lekarski. 2014 Jan;36(211):28-30.
8
[Stiff-person syndrome: a case report].[僵人综合征:一例报告]
Rev Neurol. 2008;47(5):249-52.
9
Stiff limb syndrome: end of spectrum or a separate entity?僵肢综合征:谱系末端还是独立实体?
Pain Med. 2009 Apr;10(3):594-7. doi: 10.1111/j.1526-4637.2009.00578.x. Epub 2009 Mar 19.
10
[Successful treatment in a patient with a focal form of stiff-person syndrome using plasma exchange and intravenous immunoglobulin therapy].[采用血浆置换和静脉注射免疫球蛋白疗法成功治疗一例局灶型僵人综合征患者]
Rinsho Shinkeigaku. 2002 Aug;42(8):766-70.

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In thrombin stimulated human platelets Citalopram, Promethazine, Risperidone, and Ziprasidone, but not Diazepam, may exert their pharmacological effects also through intercalation in membrane phospholipids in a receptor-independent manner.在凝血酶刺激的人血小板中,西酞普兰、异丙嗪、利培酮和齐拉西酮可能通过以受体非依赖方式插入膜磷脂来发挥其药理作用,但地西泮并非如此。
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