Doi Shinichiro, Kimura Shuhei, Morizane Yuki, Hosogi Mika, Hosokawa Mio, Shiode Yusuke, Kawata Tetsuhiro, Kondo Eisei, Shiraga Fumio
Department of Ophthalmology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.
Acta Med Okayama. 2014 Dec;68(6):379-83. doi: 10.18926/AMO/53028.
We report a case of POEMS syndrome in a 20-year-old patient diagnosed after visiting an eye clinic with a chief complaint of reduced visual acuity. A male university student aged 20 years was referred to our department complaining of blurred vision in both eyes that had persisted for 1 month. He also noted headache, nausea, and paresthesia in the lower extremities around the same time. The visual acuity of his right and left eye was 20/40 and 20/20, respectively. Optic disc edema and serous retinal detachment were present. Brain magnetic resonance imaging showed no intracranial abnormalities, while elevated cerebrospinal fluid pressure, reduced nerve conduction velocity in both lower extremities, hepatosplenomegaly, M proteinemia, high blood VEGF levels, osteoblastic and osteolytic changes in the spine, and atypical plasma cells in bone lesions were noted. From the above findings, the patient was diagnosed with POEMS syndrome. He received high-dose dexamethasone, thalidomide, and radiotherapy on the sacral mass, followed by high-dose melphalan with autologous stem-cell support, and showed subsequent systemic and ophthalmologic improvement. Here, we report the youngest case ever of POEMS syndrome with ocular manifestation. If patients have optic disc edema in both eyes with no intracranial space-occupying lesion, POEMS syndrome should be considered in differential diagnosis, regardless of age.
我们报告一例20岁的POEMS综合征患者,该患者因视力下降为主诉就诊眼科诊所后被确诊。一名20岁的男大学生因双眼视力模糊持续1个月转诊至我科。他同时还注意到头痛、恶心以及下肢感觉异常。他右眼和左眼的视力分别为20/40和20/20。存在视盘水肿和浆液性视网膜脱离。脑部磁共振成像未显示颅内异常,但脑脊液压力升高、双下肢神经传导速度减慢、肝脾肿大、M蛋白血症、血液VEGF水平升高、脊柱有成骨和溶骨改变以及骨病变中有非典型浆细胞。根据上述发现,该患者被诊断为POEMS综合征。他接受了大剂量地塞米松、沙利度胺以及骶尾部肿块的放射治疗,随后接受了大剂量美法仑联合自体干细胞支持治疗,随后全身及眼部情况均有改善。在此,我们报告有史以来最年轻的有眼部表现的POEMS综合征病例。如果患者双眼出现视盘水肿且无颅内占位性病变,无论年龄大小,鉴别诊断时均应考虑POEMS综合征。