Satow T, Nabeshima S, Yamazoe N, Isaka F, Motoyama Y, Higuchi K, Kawamura Y, Hayashino Y
Department of Neurosurgery, Tenri Hospital, Japan.
No Shinkei Geka. 2000 Nov;28(11):1023-8.
A case of intravascular malignant lymphomatosis (IML) presenting as progressive cerebral infarction is reported. A 62-year-old previously healthy male developed progressive dementia. MRI of the brain at the nearest hospital revealed multiple infarcts with unknown etiology. His level of consciousness deteriorated rapidly, and then he was transferred to our hospital for further evaluation. High grade fever, raised serum C reactive protein (CRP), and raised lymphoma markers (serum LDH and soluble IL-2 receptor (sIL-2R)) were observed. Repeated brain MRI disclosed progression of multifocal cerebral infarctions. We considered IML most likely, and we performed muscle biopsy. However muscle biopsy didn't demonstrate any proliferation of neoplastic cells of lymphoid origin within small vessels. Thereafter IML was diagnosed by brain biopsy. The patient underwent chemotherapy, but died of pneumonia due to severe myelosuppression. IML is a rare disease but most commonly shows neurological symptomatology as its clinical manifestation. Dementia is the most common neurological symptom, and progressive multiple infarction is the most common of the MRI findings. Rapidly progressive dementia associated with multiple infarction, when elevated CRP, LDH and sIL-2R are observed in the laboratory data, is suggestive of IML.
报告了一例表现为进行性脑梗死的血管内恶性淋巴瘤病(IML)病例。一名62岁既往健康的男性出现进行性痴呆。最近一家医院的脑部MRI显示多发梗死灶,病因不明。他的意识水平迅速恶化,随后被转至我院进一步评估。观察到高热、血清C反应蛋白(CRP)升高以及淋巴瘤标志物(血清乳酸脱氢酶和可溶性白细胞介素-2受体(sIL-2R))升高。重复脑部MRI显示多灶性脑梗死进展。我们高度怀疑为IML,并进行了肌肉活检。然而,肌肉活检未显示小血管内有任何淋巴源性肿瘤细胞增殖。此后通过脑活检确诊为IML。患者接受了化疗,但因严重骨髓抑制死于肺炎。IML是一种罕见疾病,但其临床表现最常见的是神经系统症状。痴呆是最常见的神经系统症状,进行性多发梗死是MRI最常见的表现。当实验室数据中观察到CRP、LDH和sIL-2R升高时,与多发梗死相关的快速进行性痴呆提示为IML。