Faculty of Medicine, Department of Radiology, Selcuk University, Konya, Turkey.
Br J Neurosurg. 2023 Jun;37(3):416-419. doi: 10.1080/02688697.2020.1817318. Epub 2020 Sep 10.
Fahr's disease, also known as familial idiopathic basal ganglia calcification or bilateral strio-pallido-dentate calcinosis, is a rare entity characterized by abnormal vascular calcium depositionin the thalamus, basal ganglia, cerebral cortex and the dentate nuclei of the cerebellum. Intracranial ependymomas comprise approximately 2% to 9% of all neuroepithelial tumors. It is reported that supratentorial ependymoma constitute 30% to 50% of all intracranial ependymal tumors. Among supratentorial ependymomas, approximately 50% of them are located extraventricular and demonstrate no relationship with the ventricularsystem.The association of brain tumor with Fahr's disease is a rare entity and has been reported several times before. Whereas, to best our knowledge, the association of Fahr's disease and supratentorial anaplastic ependymoma is described in the present study for the first time.
Fahr 病,又称家族性特发性基底节钙化或双侧纹状体苍白球齿状核钙沉积症,是一种罕见疾病,其特征是异常血管钙沉积于丘脑、基底节、大脑皮层和小脑齿状核。颅内室管膜瘤约占所有神经上皮肿瘤的 2%至 9%。据报道,幕上室管膜瘤占所有颅内室管膜瘤的 30%至 50%。在幕上室管膜瘤中,约 50%位于脑室外,与脑室系统无关系。脑肿瘤与 Fahr 病的关联是一种罕见的现象,以前曾有过几次报道。然而,据我们所知, Fahr 病和幕上间变性室管膜瘤的关联在本研究中是首次描述。