肿瘤性多发性硬化症临床模拟急性脑卒中及沿活检道病变转移一例报告

O. Tritanon, Arunee Singhsnaeh, Jiraporn Laothamatus, A. Boongird, D. Ratanakorn, P. Nitiyanant
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摘要

肿瘤性多发性硬化症是脱髓鞘疾病的一种,患者可表现为急性中风。我们报告了一例49岁女性高血压控制良好,入院前15小时出现右偏瘫。初步诊断为急性脑卒中。急诊电脑断层显示左晶状体核及左内囊后肢低密度病变。磁共振成像(MRI)显示左侧晶状体核、左侧内囊、左侧丘脑、左侧额顶叶室周区FLAIR病变高,部分区域弥散受限,增强不均匀。病变的MR谱显示胆碱峰升高,肌酸和NAA峰降低,最大胆碱/肌酸比2.25。她的症状恶化为进行性头痛和运动性失语。后续MRI显示病变沿左额叶活检道呈不均匀强化延伸,增强和MR谱模式与病变相似。开颅切除左额叶病变包括肿块并行活检道。病变表现为急性和慢性炎症细胞浸润,伴巨噬细胞、坏死组织和反应性胶质细胞增生。进一步的病理检查显示脱髓鞘灶与相对轴突保存,大量CD68+巨噬细胞与胞浆内Luxol快蓝(+)髓磷脂碎片。血管周围和实质的CD3+ t细胞被发现,特别是脱髓鞘灶。这些发现支持肿瘤性多发性硬化症的诊断。经甲泼尼龙脉冲治疗和静脉注射免疫球蛋白(IVIG)治疗后病情好转。治疗后4个月的MRI随访显示先前存在的不均匀强化病变几乎消失。
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Tumefactive Multiple Sclerosis Clinically Mimicking Acute Stroke and Lesional Migration Along the Biopsy Tract: A Case Report
Tumefactive multiple sclerosis is a form of demyelinating disease which patient can present with acute stroke. We reported a case of a 49-year-old woman with well controlled hypertension, who presented with right hemiplegia 15 hours prior to admission. The initial diagnosis of acute stroke was made. Emergency computed tomography showed hypodense lesion at the left lentiform nucleus and posterior limb of the left internal capsule. The magnetic resonance imaging (MRI) study showed hyperintense FLAIR lesion in the left lentiform nucleus, left internal capsule, left thalamus, and periventricular area of the left frontoparietal region, some areas of restricted diffusion and inhomogeneous enhancement. The MR spectroscopy (MRS) of the lesion showed increased choline peak, decreased creatine and NAA peaks, and maximal choline to creatine ratio 2.25. Her symptoms deteriorated with progressive headache and motor aphasia. The follow up MRI showed extension of the inhomogeneous enhancing lesion along the biopsy tract at the left frontal lobe with the enhancing and MR spectra pattern similar to the lesion. The craniotomy with left frontal lesion excision included the mass and the biopsy tract was done. The lesion showed acute and chronic inflammatory cell infiltration with macrophages, necrotic tissue and reactive gliosis. The further pathological worked up demonstrated foci of demyelination with relative axonal preservation, numerous CD68+ macrophages with intracyto-plasmic Luxol fast blue(+) myelin debris. Perivascular and parenchymal CD3+ T-cells were identified, especially in demyelinating foci. These findings supported the diagnosis of tumefactive multiple sclerosis. Her conditions were improved after treating with pulse methylprednisolone and intravenous immunoglobulin (IVIG). Follow up MRI study 4 months after treatment revealed almost resolution of the preexisting inhomogeneous enhancing lesion.
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