背景:复发或复发性根瘤性脱髓鞘很少见,除了个别病例报告外,还没有研究过。
目的:我们检查了临床病程,神经影像学,脑脊液(CSF),复发性肿瘤性脱髓鞘病变(TDL)患者的治疗和预后。
方法:我们使用PubMed来识别复发性TDL的报告,并包括其他细节,未发表的患者。
结果:我们确定了18例(11F,7米)。指数TDL发作的中位年龄为37岁(范围12-72),大多数为孤立性病变72%(13/18)。在25%(4/16)中检测到CSF限制性寡克隆带(OCB)。只有一个测试的人(n=13)对AQP4-IgG呈阳性。中度至显著的治疗反应(高剂量皮质类固醇,有或没有额外的血浆置换,IVIg或疾病改善疗法)在89%的治疗患者中很明显。中位随访36个月(范围6-144)时的EDSS中位数为2(范围1-10)。大多数人仍在门诊(EDSS<4in13/18),但有1名患者死亡。
结论:复发性TDL患者的中位年龄与典型MS相似,但差异包括较低的女性:男性性别比例,较大的病变,和相对缺乏CSF限制的OCB。该组患者的结果各不相同,从最小残疾到死亡。
BACKGROUND: Relapsing or recurrent tumefactive demyelination is rare and has not been studied beyond individual case reports.
OBJECTIVE: We examined the clinical course, neuroimaging, cerebrospinal fluid (CSF), treatment and outcomes of patients with recurrent tumefactive demyelinating lesions (TDLs).
METHODS: We used PubMed to identify reports of recurrent TDLs and included the details of an additional, unpublished patient.
RESULTS: We identified 18 cases (11F, 7 M). The median age at onset of the index TDL was 37 years (range 12-72) and most were solitary lesions 72 % (13/18). CSF-restricted oligoclonal bands (OCBs) were detected in 25 % (4/16). Only one of those tested (n = 13) was positive for AQP4-IgG. A moderate-to-marked treatment response (high dose corticosteroid with or without additional plasmapheresis, IVIg or disease modifying therapies) was evident in 89 % of treated patients. Median EDSS at the median follow-up of 36 months (range 6-144) was 2 (range 1-10). Most remained ambulatory (EDSS < 4 in 13/18), but 1 patient died.
CONCLUSIONS: The median age of patients with relapsing TDLs is similar to that of typical MS, but differences include a lower female:male sex ratio, larger lesions, and a comparative lack of CSF-restricted OCBs. Outcomes vary among this group of patients ranging from minimal disability through to death.