Bajantri Bharat, Venkatram Sindhaghatta, Niazi Masooma, Singh Tushi, Diaz-Fuentes Gilda
Division of Pulmonary and Critical Care, Department of Medicine Department of Medicine Department of Pathology, Bronx Lebanon Hospital Center, Bronx, NY, USA.
Medicine (Baltimore). 2017 Nov;96(45):e8516. doi: 10.1097/MD.0000000000008516.
Neurosarcoidosis (NS) is an uncommon manifestation of systemic sarcoidosis, with a propensity for middle-agedwomen. Often discovered only at autopsy, rates of neurologic involvement (5%-10%) reported in the literature underscore a lack of sensitivity and specificity in current diagnostic methods.
Herein, we describe a 53-year-old woman who presented with gait imbalance and distal extremity muscular weakness. She was known to harbor a brain mass (4 years in duration) that was monitored and recently seemed to enlarge.
A subsequent brain biopsy showed necrotizing granulomatous inflammation suggestive of NS. However, no clinical or radiologic evidence of activity was found in other organs.
Ultimately, endo and transbronchial biopsies were performed, providing histologic confirmation of systemic sarcoidosis.
This approach is advised in all instances of suspected NS where systemic involvement is in question.
神经结节病(NS)是系统性结节病的一种罕见表现形式,好发于中年女性。通常仅在尸检时才被发现,文献报道的神经系统受累率(5%-10%)凸显了当前诊断方法缺乏敏感性和特异性。
在此,我们描述一位53岁女性,她出现步态不稳和远端肢体肌肉无力。已知她有一个脑肿块(持续4年),一直在监测,最近似乎增大了。
随后的脑活检显示坏死性肉芽肿性炎症,提示为NS。然而,在其他器官未发现临床或影像学活动证据。
最终,进行了经支气管内活检和经支气管活检,提供了系统性结节病的组织学确诊依据。
对于所有怀疑有系统性受累的NS病例,建议采用这种方法。