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目的探讨伴钙化和囊变的脑白质病(LCC)的临床、影像、病理学特点及其诊断与鉴别诊断。方法对1例脑白质病患者行头颅CT和MRI扫描,抽取脑脊液检测囊虫抗体及血清钙、磷、甲状旁腺素,眼底和膝关节X线检查,并手术切除相关囊性病灶行病理学检查,同时对其父母行眼底和头颅CT等检查。结果神经系统专科检查示病变定位于右侧皮质脊髓束。CT平扫示脑内广泛钙化并伴囊性灶(位于双侧大脑半球、丘脑、基底节区及左侧脑室内);MRI增强扫描示囊性灶边缘轻度环状强化。组织病理学示脑组织内大小不等的囊性扩张区伴大量钙化和含铁血黄素沉积;少数区域可见小血管炎改变伴管壁纤维素样变性和血栓形成;局部呈血管瘤样增生,管腔充血、扩张;脑组织胶质细胞增生,Rosenthal纤维和嗜酸小体形成。患者脑脊液、实验室生化检查、眼底及右膝X线检查等均正常;对其父母的各项检查也均正常。结论 LCC的诊断需影像学、临床特点和组织学三结合,但其影像学上钙化形态和不对称分布的特点具有重要诊断意义。目前认为脑白质病是一种闭塞性脑微血管疾病,但其病因尚不明确;进一步的治疗方案尚存在争议。
Objective To investigate the clinical, imaging and pathological characteristics of leukoplakia with calcification and cystic degeneration (LCC) and their diagnostic and differential diagnosis. Methods One patient with leukoencephalopathy underwent CT scan and MRI scan. The cerebrospinal fluid was collected for the detection of cysticercosis antibody and serum calcium, phosphorus, parathyroid hormone, fundus and knee joint X-ray examination. Surgical resection of cystic lesions was performed. Check, while his parents line of sight and head CT and other tests. Results The neurological examination showed that the lesions located in the right corticospinal tract. CT scan shows a wide range of brain calcification with cystic lesions (located in the bilateral cerebral hemispheres, thalamus, basal ganglia and left ventricle); MRI enhanced cystic lesions showed mild edge enhancement. Histopathology showed cystic dilatation of varying sizes within the brain tissue with a large number of calcifications and hemosiderin deposition; a small number of areas can be seen with changes in vascular wall fibrosis and thrombosis of small vasculitis; hemangiomatous hyperplasia, Lumen hyperemia and dilation; glial cell proliferation in brain tissue, formation of Rosenthal fibers and eosinophils. Patients with cerebrospinal fluid, laboratory biochemical tests, fundus and right knee X-ray examination were normal; the inspection of their parents are also normal. Conclusion The diagnosis of LCC requires the combination of imaging, clinical features and histology. However, the imaging features of calcifications and asymmetric distribution are of important diagnostic significance. Leukodystrophy is currently considered an occlusive cerebral microvascular disease, but the etiology is not yet clear; further treatment is still controversial.