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中枢神经系统血管内淋巴瘤:快速进展性痴呆的一个未被充分认识的病因。

CNS intravascular lymphoma: an underappreciated cause of rapidly progressive dementia.

作者信息

Panda Akhila Kumar, Malik Seema

机构信息

Department of Neurology, Institute of Human Behaviour and Allied Sciences (IHBAS), Delhi, India.

出版信息

BMJ Case Rep. 2014 Apr 10;2014:bcr2014203772. doi: 10.1136/bcr-2014-203772.

Abstract

Intravascular lymphoma is a rare subtype of extranodal large cell non-Hodgkin's lymphoma that is usually seen in the elderly. It can occasionally present with neurological symptoms in the form of dementia, focal neurological deficit and seizure. Diagnosis is difficult because of non-specific clinical manifestation. We report a case of a 38-year-old woman presenting with rapidly progressive dementia and seizure. MRI of the brain showed bilateral diffuse involvement of cortex and subcortical white matter. Brain biopsy disclosed the aetiological confirmation of intravascular B-cell lymphoma. The patient was treated with monthly cyclophosphamide, doxorubicin, vincristine and prednisolone regimen, but unfortunately, she died after two chemotherapy cycles. So, high index of suspicion is warranted to diagnose and treat the condition early to have a better outcome.

摘要

血管内淋巴瘤是结外大细胞非霍奇金淋巴瘤的一种罕见亚型,多见于老年人。它偶尔会以痴呆、局灶性神经功能缺损和癫痫发作的形式出现神经症状。由于临床表现不具特异性,诊断较为困难。我们报告一例38岁女性患者,表现为快速进展性痴呆和癫痫发作。脑部MRI显示双侧皮质和皮质下白质弥漫性受累。脑活检确诊为血管内B细胞淋巴瘤。该患者接受了每月一次的环磷酰胺、阿霉素、长春新碱和泼尼松龙方案治疗,但不幸的是,她在两个化疗周期后死亡。因此,需要高度怀疑以早期诊断和治疗该病,从而获得更好的预后。

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Rapidly progressive dementia: prion diseases and other rapid dementias.快速进展性痴呆:朊病毒病及其他快速进展性痴呆
Continuum (Minneap Minn). 2010 Apr;16(2 Dementia):31-56. doi: 10.1212/01.CON.0000368211.79211.4c.

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