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目的 分析我院 8例原发进展型多发性硬化 (PPMS)患者的临床特征。方法 符合Lublin等PPMS诊断标准者纳入此研究。结果 收集了 8例PPMS患者的临床资料 ,其中男性 1例 ,女性 7例 ,年龄 32～ 62 ( 39 75± 9 91)岁 ,并结合实验室检查进行了分析。本研究中的 8例PPMS患者在不同类型MS构成比例、发病年龄、起病症状、MRI检查、诱发电位、脑脊液免疫学检查、治疗等方面与其他文献报道基本一致。但是本研究中PPMS女性较多。另外 ,1例在入院前曾误诊为运动神经元疾病 ,另 1例曾误诊为颅内转移瘤 ,经术后病理证实为脱髓鞘病变。结论 PPMS在MS中较为少见 ,应与其他神经系统疾病进行鉴别
Objective To analyze the clinical features of 8 patients with primary progressive multiple sclerosis (PPMS) in our hospital. Methods Patients who met the criteria of Lublin et al. PPMS were included in this study. Results The clinical data of 8 patients with PPMS were collected, including 1 males and 7 females, ranging in age from 32 to 62 (39 75 ± 9 91) years and analyzed in combination with laboratory tests. 8 cases of PPMS patients in this study in different types of MS composition ratio, age of onset, onset symptoms, MRI examination, evoked potential, immunological examination of cerebrospinal fluid, treatment and other aspects of the same with other reports. However, there are more women with PPMS in this study. In addition, one patient had been misdiagnosed as motor neuron disease before admission, and the other one had been misdiagnosed as intracranial metastasis, which was confirmed by pathology as demyelinating disease. Conclusion PPMS is rare in MS and should be differentiated from other neurological diseases