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法尔综合征与慢性淋巴细胞性甲状腺炎。

The Fahr syndrome and the chronic lymphocytic thyroiditis.

作者信息

Sava Anca, Dumitrescu Gabriela, Haba Danisia, Hodorog Diana, Mihailov Claudia, Şapte Elena

机构信息

Discipline of Anatomy and Embryology, Morphofunctional Department, "Grigore T. Popa" University of Medicine and Pharmacy, Iassy, Romania.

出版信息

Rom J Morphol Embryol. 2013;54(1):195-200.

Abstract

Fahr syndrome (FS) refers to basal ganglia calcification that is associated with many neurological and psychiatric abnormalities and appears as secondary to other diseases. We described a case of FS patient who was admitted in the Department of Neurology of "Prof. Dr. Nicolae Oblu" Clinical Emergency Hospital, Iassy, Romania, with seizure and mood disorders. On CT, the cause of seizure was found to be the bilateral calcifications of cerebellum, basal ganglia, thalamus and internal capsule. As the patient died after 15 days of hospitalization due to new seizures and gastrointestinal infection, an autopsy was made. Grossly, there were bilateral symmetrically gritty yellow areas in basal ganglia, thalami, internal capsule, cerebral cortex, cerebellar folia, dentate nucleus, and brain stem. A detailed histopathological examination revealed five types of calcium deposits within the walls of capillaries, small and medium-sized arteries from the intracerebral affected areas, chronic lymphocytic thyroiditis and fibro-adipose tissue instead of parathyroids. We consider that intracerebral symmetrical calcifications were the results of the hypoparathyroidism determined by an ancient autoimmune parathyroiditis that evolved to fibrosis as at microscopy we found an autoimmune thyroiditis.

摘要

Fahr综合征(FS)是指基底节钙化,与许多神经和精神异常相关,且表现为继发于其他疾病。我们描述了一例FS患者,该患者因癫痫发作和情绪障碍入住罗马尼亚雅西“尼古拉·奥布卢教授”临床急诊医院神经内科。CT检查发现,癫痫发作的原因是小脑、基底节、丘脑和内囊的双侧钙化。由于患者在住院15天后因新的癫痫发作和胃肠道感染死亡,故进行了尸检。大体检查发现,基底节、丘脑、内囊、大脑皮质、小脑叶片、齿状核和脑干有双侧对称的沙砾样黄色区域。详细的组织病理学检查显示,在脑内受累区域的毛细血管、中小动脉壁内有五种类型的钙沉积,存在慢性淋巴细胞性甲状腺炎以及取代甲状旁腺的纤维脂肪组织。我们认为,脑内对称性钙化是由一种古老的自身免疫性甲状旁腺炎导致的甲状旁腺功能减退的结果,这种炎症在显微镜下发展为纤维化,因为我们发现了自身免疫性甲状腺炎。

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