Goel Jitesh, Anadure Ravi, Gupta Salil, Wilson Vinny, Saxena Rajeev, Sahu Samaresh, Mutreja Deepti
Department of Neurology, Command Hospital Air Force, Bengaluru, Karnataka, India.
Department of Medicine, Agram Post, Command Hospital Air Force, Bengaluru, Karnataka, India.
Neurol India. 2020 May-Jun;68(3):609-616. doi: 10.4103/0028-3886.288976.
Sarcoidosis is an inflammatory granulomatous disease affecting multiple organ systems. Neurological manifestations are rare and seen in approximately 5% cases of sarcoidosis. They may commonly precede the diagnosis of sarcoidosis. Since there is paucity of Indian literature on this subject, we decided to review the clinical and radiological profile, laboratory abnormalities, treatment and long-term outcomes in our patients with neurosarcoidosis (NS).
The study was done by retrospective review of medical records for all cases diagnosed as NS during the period Jan 2014-Jan 2018. These cases were classified as definite, probable, and possible NS, on the basis of established diagnostic parameters (Zajicek criteria). The follow-up record in these cases ranged from 6 months to 3 years, with special emphasis on monitoring the response to treatment and long-term disability.
The cases showed varied clinical abnormalities and imaging findings. Cranial neuropathies and myelopathy were the most common clinical presentations. Optic neuritis was most common cranial neuropathy, followed by facial nerve palsy and lower cranial nerve palsies. Most common magnetic resonance imaging findings were T2 hyperintense parenchymal lesions and meningeal enhancement. There was strong correlation between baseline clinico radiological parameters and long-term outcomes, as evidenced by relatively poor prognosis seen in cases with bilateral optic neuritis, myelopathy and imaging evidence of hydrocephalus, or leptomeningitis.
The diagnosis of NS requires a high degree of suspicion, coupled with exclusion of alternate diagnosis. It commonly precedes the onset of systemic sarcoidosis. Central nervous system involvement in sarcoidosis is associated with poor clinical outcomes.
结节病是一种影响多个器官系统的炎症性肉芽肿疾病。神经系统表现较为罕见，约5%的结节病患者会出现。它们可能常在结节病确诊之前出现。由于关于该主题的印度文献较少，我们决定回顾我们的神经结节病（NS）患者的临床和放射学特征、实验室异常、治疗及长期预后情况。
本研究通过回顾性分析2014年1月至2018年期间所有诊断为NS的病例的病历进行。根据既定的诊断参数（扎伊切克标准），这些病例被分为确诊、可能和疑似NS。这些病例的随访记录为6个月至3年，特别强调监测治疗反应和长期残疾情况。
这些病例表现出多样的临床异常和影像学表现。颅神经病变和脊髓病是最常见的临床表现。视神经炎是最常见的颅神经病变，其次是面神经麻痹和低位颅神经麻痹。最常见的磁共振成像表现为T2高信号实质病变和脑膜强化。基线临床放射学参数与长期预后之间存在很强的相关性，双侧视神经炎、脊髓病以及有脑积水或软脑膜炎影像学证据的病例预后相对较差，这证明了这一点。
NS的诊断需要高度怀疑，并排除其他诊断。它通常在系统性结节病发作之前出现。结节病累及中枢神经系统与不良临床预后相关。
