Hematology and Oncology, The University of Texas Health Science Center at Houston, Houston, Texas, USA.
Hematology and Oncology, The University of Texas Health Science Center at Houston, Houston, Texas, USA
BMJ Case Rep. 2022 Jul 7;15(7):e250639. doi: 10.1136/bcr-2022-250639.
Stiff person syndrome (SPS) is a rare, debilitating neurological illness characterised by rigidity and spasms of the axial muscles, causing severe restrictions to mobility. SPS can be classic, partial or paraneoplastic. We report a case of a young woman who presented with seizures and painful spasms of the thoracolumbar muscles who was subsequently diagnosed with SPS. Serological work revealed glutamic acid decarboxylase (GAD) antibodies and imaging showed a large mediastinal mass. The patient underwent surgical resection of the mediastinal mass and final pathology revealed well-differentiated mediastinal liposarcoma. She received five sessions of plasma exchange and her neurological symptoms gradually improved after surgery. This case highlights a rare case of GAD antibody-positive paraneoplastic SPS associated with mediastinal liposarcoma.
僵人综合征（SPS）是一种罕见的使人虚弱的神经系统疾病，其特征为脊柱肌肉的僵硬和痉挛，导致严重的活动受限。SPS 可分为典型、部分性和副肿瘤性。我们报告了一例年轻女性，她以癫痫发作和胸腰椎肌肉疼痛性痉挛为表现，随后被诊断为 SPS。血清学检查发现谷氨酸脱羧酶（GAD）抗体，影像学检查显示纵隔巨大肿块。患者接受了纵隔肿块的手术切除，最终的病理显示为分化良好的纵隔脂肪肉瘤。她接受了五次血浆置换，术后神经症状逐渐改善。本病例提示了一种罕见的与纵隔脂肪肉瘤相关的 GAD 抗体阳性副肿瘤性 SPS。
