Department of Neurology and Psychiatry, Neurosurgery, 'Sapienza' University of Rome, Viale del Policlinico 155, 00146, Rome, Italy.
Department of Radiological and Pathological Science, 'Sapienza' University of Rome, Rome, Italy.
Acta Neurol Belg. 2021 Apr;121(2):415-420. doi: 10.1007/s13760-019-01189-1. Epub 2019 Jul 11.
Sarcoidosis is a rare systemic disease characterized by growth and organization of inflammatory cells in a granuloma. Granulomas can localize in any parts of the human body. The main localization is represented by lungs, lymph nodes, eyes and skin. Any organ, however, can be affected. Central nervous system (CNS) represents a rare localization of sarcoidosis, in fact, only 1% of patient with sarcoidosis present brain and/or spinal cord localization of the granulomas associated with this disease. This condition takes the name of Neurosarcoidosis. Its diagnosis and management pose a significant challenge, as its clinical manifestation and appearance in imaging studies are difficult to distinguish from more common lesion of the spinal cord. In this paper, we present the case of a 45-year-old woman who presented back pain associated with neurologic signs of myelopathy. Spinal cord MRI documented a T2 hyperintense signal around medullary conus. She underwent a gross total removal of the lesion. Histopathological examination revealed spinal sarcoidosis. Next total body CT scan did not show other localization of the disease. The aim of the present paper is to report a very rare case of spinal epidural sarcoidosis, actually only five cases have been described, without other localization of the disease. The present article underlines the difference between this forms of spinal sarcoidosis compared to intradural extramedullary and intramedullary spinal sarcoidosis.
结节病是一种罕见的全身系统性疾病，其特征是炎症细胞在肉芽肿中生长和组织化。肉芽肿可以在人体的任何部位定位。主要定位是肺部、淋巴结、眼睛和皮肤。然而，任何器官都可能受到影响。中枢神经系统（CNS）是结节病的罕见定位，事实上，只有 1%的结节病患者出现与这种疾病相关的脑和/或脊髓肉芽肿的定位。这种情况被称为神经结节病。其诊断和管理带来了重大挑战，因为其临床表现和影像学研究中的表现很难与更常见的脊髓病变区分开来。在本文中，我们介绍了一位 45 岁女性的病例，她表现为背痛伴有脊髓病的神经体征。脊髓 MRI 记录了脊髓圆锥周围 T2 高信号。她接受了病变的大体全切除。组织病理学检查显示为脊髓结节病。随后的全身 CT 扫描未显示其他部位的疾病。本文的目的是报告一例非常罕见的脊髓硬膜外结节病，实际上只有五例被描述，没有其他部位的疾病。本文强调了这种脊髓结节病与硬脊膜外髓内和髓内脊髓结节病的区别。
