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Leukoencephalopathy with cerebral calcifications and cysts: clinical and pathological features in two adults

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Abstract

Two adult patients diagnosed with Leukoencephalopathy with cerebral calcifications and cysts (LCC) were presented. Both patients had a long-term (8–10 years) following-up. Radiological findings of both patients revealed the characteristic signs of LCC: cerebral white matter abnormalities, calcifications, and cysts. In case 1, the initial CT scan showed a low-density area in the right frontal lobe and it had developed into a large cystic lesion after 8 years. Histopathological determination revealed that the cyst wall was associated with hemorrhage, angiomatous formation, and some Rosenthal fibers. In case 2, a major cystic lesion was located at the left parietal lobe which was resected and an old hematoma was found inside the cyst. Nine years later, the follow-up neuroimaging of case 2 showed a remarkable improvement of white matter abnormalities and cystic lesions. Hemorrhagic fluid was observed inside the cysts. Additionally, follow-up CT and MR scans showed a rapid enlargement of cystic lesions accompanied with hemorrhagic fluid levels after a year. Then, a major cyst was surgically removed to relieve pressure symptoms. Pathology of the resected cyst exhibited an organized hemorrhage inside the cyst and a large amount of hemosiderin surrounding the cyst wall. In conclusion, our two cases demonstrated that angiomatous changes subsequent with hemorrhage may be the major mechanism of cyst formation and development.

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Acknowledgments

This work was supported by the Natural Science Foundation Program of Shandong Province (Grant No. ZR2011HQ022), Science and Technology Development Plan of Shandong Province (Grant No. 2011YD18020), and People’s Livelihood Project in Science and Technology Development Plan of Qingdao (Grant No. 14-2-3-7-nsh).

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Authors and Affiliations

  1. Department of Radiology, Affiliated Hospital of Qingdao University, No. 16 Jiangsu Road, Qingdao, 266003, China

    Xuejun Liu, Qinglan Sui & Wenjian Xu

  2. Department of Neurology, Affiliated Hospital of Qingdao University, Qingdao, 266003, China

    Xueping Zheng

  3. Department of Neuroradiology, Keck Medical Center, University of Southern California, Los Angeles, USA

    Chi-shing Zee

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  1. Xuejun Liu
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  2. Xueping Zheng
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  3. Qinglan Sui
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  4. Wenjian Xu
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  5. Chi-shing Zee
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Correspondence to Wenjian Xu.

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All authors declare that they have no financial or other conflicts of interest in relation to this research and its publication.

Ethical standards and patient consent

We declare that all human and animal studies have been approved by the Affiliated Hospital of Qingdao University and have therefore been performed in accordance with the ethical standards laid down in the 1964. Declaration of Helsinki and its later amendments: We declare that all patients gave informed consent prior to inclusion in this study.

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Liu, X., Zheng, X., Sui, Q. et al. Leukoencephalopathy with cerebral calcifications and cysts: clinical and pathological features in two adults. Acta Neurol Belg 116, 47–52 (2016). https://doi.org/10.1007/s13760-015-0501-5

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  • DOI: https://doi.org/10.1007/s13760-015-0501-5

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