The neurological masquerade of intravascular lymphomatosis
The neurological masquerade of intravascular lymphomatosis
复制标题
DOI：
10.1001/archneur.59.3.439
复制
发表时间：
2002-03-01
影响因子：
--
通讯作者：
Azzarelli, B
中科院分区：
文献类型：
--
作者：
Beristain, X;Azzarelli, B
背景资料：血管内淋巴瘤病（IVL）是一种罕见的全身性疾病，其特征是恶性淋巴瘤细胞阻塞小血管。中枢神经系统受累通常表现为亚急性脑病，痴呆，癫痫发作，或多灶性脑血管events.Objective：为了提高认识IVL，一种不常见的原因，神经系统diseases.Design：这是一个回顾性的病例系列8例病理证实的IVL与神经系统疾病。患者是1962年4月至1998年10月期间在印第安纳州大学医学院和华盛顿武装部队病理学研究所收集的病理系列的一部分。设置：在三级转诊医院进行神经学和神经病理学检查。11例患者病理诊断为IVL，但3例因缺乏适当的临床信息而未纳入本评价。在最终的样本（n = 8）中，有4名男性和4名女性（平均+/-SD年龄，62.9+/-9.9岁）。结果：所有8例患者都有局灶性神经功能缺损，7例有脑病或痴呆，5例有癫痫发作，2例有脊髓病。死亡发生在症状发作后平均7.7个月（范围，1-24个月）。所有患者的脑脊液蛋白水平升高，4例有白细胞增多症，2例脑脊液IgG水平升高。4例谁接受了脑活检，1被诊断为有IVL前dead.Conclusions：血管内淋巴瘤病是一种罕见的疾病，无数的潜在的神经系统表现。诊断需要高度怀疑和病理检查。如果早期诊断，积极的化疗是有可能治愈的，尽管总体预后仍然很差。
Background: Intravascular lymphomatosis (IVL) is an uncommon systemic disease characterized by occlusion of small vessels by malignant lymphomatous cells. Central nervous system involvement usually presents as subacute encephalopathy, dementia, seizures, or multifocal cerebrovascular events.Objective: To increase awareness about IVL, an uncommon cause of neurological disease.Design: This is a retrospective case series of 8 pathologically proved cases of IVL with neurological disease. Patients were part of a pathological series collected between April 1962 and October 1998 at Indiana University School of Medicine and the Armed Forces Institute of Pathology, Washington, DC.Setting: Neurological and neuropathological examinations were performed at tertiary referral hospitals.Patients: Eleven patients were diagnosed pathologically as having IVL, but 3 were not included in this evaluation because of a lack of appropriate clinical information. Of the final sample (n = 8), there were 4 men and 4 women (mean+/-SD age, 62.9+/-9.9 years).Results: All 8 patients had focal neurological deficits, 7 had encephalopathy or dementia, 5 had epileptic seizures, and 2 had myelopathy. Death occurred at a mean of 7.7 months (range, 1-24 months) after the onset of symptoms. All patients had elevated cerebrospinal fluid protein levels, 4 had pleocytosis, and 2 had an elevated IgG level in their cerebrospinal fluid. Of the 4 patients who underwent a brain biopsy, 1 was diagnosed as having IVL before death.Conclusions: Intravascular lymphomatosis is an uncommon disease with a myriad of potential neurological manifestations. Diagnosis requires a high index of suspicion and a pathological examination. If diagnosed early, aggressive chemotherapy is potentially curative, although the overall prognosis remains dismal.