Broussolle E, Vighetto A, Bancel B, Confavreux C, Pialat J, Aimard G
Clinique Neurologique, Hôpital Neurologique, Lyon, France.
Clin Neurol Neurosurg. 1991;93(2):165-70. doi: 10.1016/0303-8467(91)90061-s.
The P.O.E.M.S. syndrome is a rare clinical entity that has been described mainly in Japan. It is characterized by a progressive polyneuropathy with raised CSF protein content, organomegaly, endocrinopathy, skin changes and plasma cell dyscrasia. We report a new documented case associated with a solitary plasmocytoma of the 12th thoracic vertebra. Immunopathological and ultrastructural studies of the peripheral nerve did not disclose any immune-specific changes. Surgery and radiation therapy of the plasmocytoma allowed a complete recovery, with a 5-year follow-up. This case report illustrates the need for serial full skeletal survey, including scintigraphy, in middle-aged patients with progressive polyneuropathy of obscure origin.
POEMS综合征是一种主要在日本被描述的罕见临床病症。其特征为伴有脑脊液蛋白含量升高的进行性多发性神经病、器官肿大、内分泌病、皮肤改变和浆细胞异常增生。我们报告一例新的有记录病例,该病例与第12胸椎孤立性浆细胞瘤相关。对周围神经的免疫病理学和超微结构研究未发现任何免疫特异性改变。对浆细胞瘤进行手术和放射治疗后实现了完全康复,并进行了5年随访。该病例报告表明,对于病因不明的进行性多发性神经病的中年患者,需要进行包括闪烁扫描在内的系列全骨骼检查。