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一例白质脑病、脑钙化和囊肿病例。

A case of leukoencephalopathy, cerebral calcifications and cysts.

作者信息

Gulati Ajay, Singh Paramjeet, Ramanathan Subramaniyan, Khandelwal N

机构信息

Department of Radiodiagnosis and Imaging, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

出版信息

Ann Indian Acad Neurol. 2011 Oct;14(4):310-2. doi: 10.4103/0972-2327.91964.

Abstract

Triad of leukoencephalopathy, cerebral calcifications and cysts (LCC) is a recently reported rare disease named 'Labrune syndrome' after the first case was reported in 1996 by Labrune et al. Herein, we report a case of a 36-year-old man with mild right-sided weakness and seizures for 5 years. CT of brain revealed extensive calcification involving bilateral basal ganglia, right thalamus and bilateral deep cerebellar nuclei. A supratentorial cystic lesion with blood fluid level was seen in left occipitotemporal region. MRI examination revealed diffuse symmetric white matter hyperintensity suggesting leukoencephalopathy. On follow-up, patient reported improvement in the weakness and no further seizure episodes. However, follow-up of MRI revealed persistence of lesions. Differential diagnosis considered were parasitic infections (hydatid, cysticercosis), Coat's plus disease and causes of diffuse cerebral calcification like Fahr's disease and post-radiotherapy/chemotherapy. Serology for parasitic infections was negative. No history of radiotherapy or chemotherapy in the past could be elicited in the history. Another close differential is Coat's plus disease which can mimic LCC pathologically.

摘要

脑白质病、脑钙化和囊肿三联征(LCC)是一种最近报道的罕见疾病,1996年Labrune等人报道首例病例后,被命名为“Labrune综合征”。在此,我们报告一例36岁男性,有5年右侧轻度无力和癫痫发作病史。脑部CT显示双侧基底节、右侧丘脑和双侧小脑深部核团广泛钙化。左侧枕颞区可见一个有液平的幕上囊性病变。MRI检查显示弥漫性对称性白质高信号,提示脑白质病。随访时,患者报告无力症状有所改善,且未再有癫痫发作。然而,MRI随访显示病变持续存在。考虑的鉴别诊断包括寄生虫感染(包虫病、囊尾蚴病)、科茨加综合征以及 Fahr病和放疗/化疗后等导致弥漫性脑钙化的病因。寄生虫感染血清学检查为阴性。病史中未发现过去有放疗或化疗史。另一个相近的鉴别诊断是科茨加综合征,其在病理上可类似LCC。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/354f/3271476/4b73dffeebe2/AIAN-14-310-g001.jpg

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