Vogels R L, van Orshoven N P, de Koning-Tijssen M A, Wouda E J
St. Lucas-Andreas Ziekenhuis, afd. Neurologie, Postbus 9243, 1006 AE Amsterdam.
Ned Tijdschr Geneeskd. 2003 Jun 21;147(25):1228-32.
In two patients, a man aged 54 years and a woman aged 49 years, stiff-person syndrome was diagnosed. This is a rare disorder of the central nervous system, with signs of an autoimmune pathogenesis. Patients present with pain and stiffness of the lower back, a complaint that is regularly seen in general practice. Moreover, the disease causes hypertonia and very painful cramps of the lower back and legs. Electromyographic examination in the resting condition reveals continuous muscle activity in the long back muscles, which decreases following the administration of diazepam. In 60% of patients, antibodies to glutamic acid decarboxylase may be found in the serum or cerebrospinal fluid; this enzyme is involved in the production of the inhibiting neurotransmitter gamma-aminobutyric acid. Both patients were treated with diazepam, baclofen and corticosteroids. Stiff-person syndrome is a rare but treatable disorder that should be considered when patients present with stiffness and pain in the lower back and upper legs.
在两名患者中,一名54岁男性和一名49岁女性,被诊断患有僵人综合征。这是一种罕见的中枢神经系统疾病,具有自身免疫发病机制的迹象。患者表现为下背部疼痛和僵硬,这是全科医疗中经常见到的主诉。此外,该疾病会导致高张力以及下背部和腿部非常疼痛的痉挛。静息状态下的肌电图检查显示,背部深层肌肉存在持续的肌肉活动,给予地西泮后这种活动会减少。在60%的患者中,血清或脑脊液中可能会发现谷氨酸脱羧酶抗体;这种酶参与抑制性神经递质γ-氨基丁酸的产生。两名患者均接受了地西泮、巴氯芬和皮质类固醇治疗。僵人综合征是一种罕见但可治疗的疾病,当患者出现下背部和大腿僵硬及疼痛时应予以考虑。