摘要
目的探讨成人变异型Dandy-Walker综合征(DWS)合并原发性脑膜黑色素瘤病的临床特点。方法回顾性分析1例成人变异型DWS合并有原发性脑膜黑色素瘤病的患者的临床资料。结果本例为19岁男性,出生后智能发育较同龄人迟缓,2018年开始反复出现头痛、恶心、呕吐,1年后症状加重,行头颅MRI平扫示DWS变异型,行脑室腹腔分流术。术后1年出现精神行为异常,复查头颅MRI示软脑膜广泛伴脑内多个结节样异常信号,MRI增强示软脑膜广泛高信号伴结节样异常信号,腰穿CSF细胞学发现黑色素细胞。结论DWS与原发性脑膜黑色素瘤病可存在共病关系,二者之间的发病机制是否存在重叠目前尚缺乏相关证据。对于这两种疾病的治疗,目前国内外尚无规范化的治疗方案。
Objective To investigate the clinical features of adult variant Dandy-Walker syndrome(DWS)complicated with primary meningeal melanoma.Methods Clinical data of a patient with adult variant DWS complicated with primary meningeal melanoma were retrospectively analyzed.Results The patient was a 19-year-old male with slower intellectual development than his peers after birth.He started to have headaches,nausea and vomiting repeatedly in 2018,and his symptoms got worse one year later.The cranial MRI scan showed the variant of DWS,and he was operated by ventriculoperitoneal shunt.After 1 year,he had abnormal mental behavior,MRI showed extensive pia mater with multiple nodular abnormal signals in the brain,MRI enhancement showed extensive high signal in pia mater with nodular abnormal signal,CSF cytology discovered melanocytes.Conclusions DWS and primary meningeal melanoma may have a comorbidity relationship.Whether there is an overlap in the pathogenesis between them is still lacking relevant evidence.For the treatment of these two diseases,there is no standardized consensus at home or abroad.
作者
黄琳
余传勇
葛剑青
HUANG Lin;YU Chuan-yong;GE Jian-qing(Department of Neurology,Brain Hospital Affiliated to Nanjing Medical University,Nanjing 210029,China)
出处
《临床神经病学杂志》
CAS
2023年第1期56-59,共4页
Journal of Clinical Neurology
