Razmeh Saeed, Habibi Amir Hasan, Sina Farzad, Alizadeh Elham, Eslami Monireh
Department of Neurology, Rasoul Akram Hospital, Iran University of Medical Sciences, Tehran, Iran.
Caspian J Intern Med. 2017 Fall;8(4):329-331. doi: 10.22088/cjim.8.4.329.
Stiff person syndrome (SPS) is a rare neurological disease resulting in stiffness and spasm of muscles. It initially affects the axial muscles and then spread to limb muscles. Emotional stress exacerbated the symptoms and signs of the disease. The pathophysiology of the disease is caused by the decreased level of the glutamic acid decarboxylase (GAD) activity due to an autoantibody against GAD that decreases the level of gamma-aminobutyric acid (GABA). In this paper, we present a case of atypical presentation of SPS with lower limb stiffness misdiagnosed as conversion disorder.
We report a patient with atypical presentation of SPS with lower limb stiffness and gait disorder misdiagnosed as conversion disorder for a year. Her antithyroid peroxidase antibody (anti-TPO Ab) level was 75 IU (normal value: 0-34 IU). Intravenous immunoglobulin (IVIG) was administered (2gr/kg, 5 days) for the patient that showed significant improvement in the follow-up visit.
It is essential that in any patient with bizarre gait disorder and suspicious to conversion disorder due to the reversibility of symptoms, SPS and other movement disorder should be considered.
僵人综合征（SPS）是一种罕见的神经系统疾病，可导致肌肉僵硬和痉挛。它最初影响躯干肌肉，随后蔓延至四肢肌肉。情绪应激会加重该病的症状和体征。该病的病理生理学是由一种针对谷氨酸脱羧酶（GAD）的自身抗体导致GAD活性水平降低，进而使γ-氨基丁酸（GABA）水平降低所致。在本文中，我们报告了一例以下肢僵硬为非典型表现的SPS病例，该病例曾被误诊为转换障碍。
我们报告了一名患有SPS非典型表现的患者，其下肢僵硬和步态障碍被误诊为转换障碍达一年之久。她的抗甲状腺过氧化物酶抗体（抗-TPO Ab）水平为75 IU（正常值：0-34 IU）。对该患者给予静脉注射免疫球蛋白（IVIG）（2gr/kg，共5天）治疗，随访时显示有显著改善。
对于任何有怪异步态障碍且因症状具有可逆性而疑似转换障碍的患者，必须考虑SPS和其他运动障碍。
