Mixed Connective Tissue Disease Presenting as Longitudinal Extensive Transverse Myelitis and Vasculitic Neuropathy

Anuj Tikoo, A. Deshpande
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Abstract

Mixed connective tissue disease (MCTD) is described as an entity with mixed features of systemic lupus erythematosus, systemic sclerosis, polymyositis/ dermatomyositis, and rheumatoid arthritis together with the presence of high-titre anti-U1 small nuclear and anti-ribonucleoprotein (anti-RNP) antibodies. Here, we present a case of an 18-year-old female patient who presented with quadriparesis, sensory loss in all four limbs, and trophic ulcers. Laboratory investigations were strongly positive for ANA, KU, SM/RNP, SM, SSA, and RIBOSOME P protein. MRI brain showed diffuse T2 hyperintensity in the spinal cord extending from cervicomedullary junction to conus with a subtle expansion of cord. A diagnosis of longitudinal extensive transverse myelitis and vasculitic neuropathy in the case of MCTD was made.
混合性结缔组织病表现为纵向广泛横贯脊髓炎和血管性神经病变
混合性结缔组织病(MCTD)被描述为具有系统性红斑狼疮、系统性硬化症、多发性肌炎/皮肌炎和类风湿性关节炎的混合特征,同时存在高滴度的抗u1小核和抗核糖核蛋白(anti-RNP)抗体。在这里,我们提出了一个18岁的女性患者,她表现为四肢瘫,四肢感觉丧失和营养性溃疡。实验室检测ANA、KU、SM/RNP、SM、SSA和核糖体P蛋白阳性。脑MRI显示脊髓弥漫性T2高信号,从颈髓交界处延伸至圆锥,伴脊髓轻度扩张。诊断纵向广泛横贯脊髓炎和血管神经病变的情况下,MCTD作出。
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