Weiss T D, Nelson J S, Woolsey R M, Zuckner J, Baldassare A R
Arthritis Rheum. 1978 Nov-Dec;21(8):982-6. doi: 10.1002/art.1780210818.
Neurologic disease is reported to occur in just 10% of patients with mixed connective tissue disease (MCTD). Most commonly, this is manifested by mild trigeminal neuralgia. This report details the clinical and neuropathologic findings of transverse myelitis in a patient with MCTD. Neurologic features include progressive areflexic paraplegia with loss of bowel and bladder function. Neuropathologically there was thinning of the thoracic cord, widespread loss of axons and myelin sheaths, reactive astrocytosis, macrophage formation, vascular thickening with perivascular chronic inflammatory cell infiltration, and calcium deposits. This case demonstrates that severe neurologic disease unresponsive to therapy can occur in MCTD.
据报道，仅10%的混合性结缔组织病（MCTD）患者会出现神经系统疾病。最常见的表现为轻度三叉神经痛。本报告详细介绍了1例MCTD患者发生横贯性脊髓炎的临床和神经病理学发现。神经学特征包括进行性无反射性截瘫伴肠道和膀胱功能丧失。神经病理学表现为胸段脊髓变薄、轴突和髓鞘广泛丧失、反应性星形细胞增生、巨噬细胞形成、血管增厚伴血管周围慢性炎性细胞浸润以及钙沉积。该病例表明，MCTD患者可能会出现对治疗无反应的严重神经系统疾病。
