Mani Arun Mathai, Prabhakar A T, Mannam Pavithra, Benjamin Rohit Ninan, Ahmed Shaikh Atif Iqbal, Mathew Donna, Singh Pankaj, Nair Aditya, Alexander P T, Vijayaraghavan Asish, Sivadasan Ajith, Mani Sunithi, Mathew Vivek, Aaron Sanjith, Alexander Mathew
Neurology Unit, Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.
Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India.
Ann Indian Acad Neurol. 2020 Jul-Aug;23(4):528-535. doi: 10.4103/aian.AIAN_638_19. Epub 2020 Jun 29.
Neurosarcoidosis (NS) is a chronic disease with a diverse clinical spectrum, therapeutic response, and outcome. There is scarce literature from our country regarding the same.
The aim of this study was to evaluate the clinical spectrum, therapeutic responses, and outcomes of NS in an Indian cohort.
In a cross-sectional study, we included all patients with NS treated at a quaternary care teaching hospital in India from January 2007 to October 2019.
Patients older than 18 years of age fulfilling the diagnostic criteria for NS from the Neurosarcoidosis Consortium Consensus Group were included in the study. The therapeutic response and the degree of disability at last follow-up were assessed.
We identified 48 patients, among them 3 were categorized as having definite NS, 30 probable NS, and 15 possible NS. Cranial neuropathy was the most common presentation (47.9%), followed by myelopathy (25%). Systemic involvement was identified in 95.83% and mediastinal lymph nodes were the most common site. Clinical improvement was seen in 65.8% and disease stabilized in 28.9%, while 5.26% deteriorated. Fifty percent recovered without any residual disability, while 26.3% had minor and 23.7% had major residual sequelae.
NS is a diverse illness, with a heterogeneous spectrum of clinical presentation, treatment response, and outcome. Cranial neuropathy is the most common presenting feature and has a good prognosis while myelopathy has an unfavorable prognosis. Meningeal and brain parenchymal disease is difficult to diagnose accurately unless systemic involvement is present. The diagnosis of NS should be clinically suspected in the appropriate clinical setting, the presence of systemic involvement should be investigated, and histologic confirmation should be attempted.
神经结节病（NS）是一种临床谱、治疗反应和预后多样的慢性疾病。我国关于此病的文献稀少。
本研究旨在评估印度队列中神经结节病的临床谱、治疗反应和预后。
在一项横断面研究中，我们纳入了2007年1月至2019年10月在印度一家四级医疗教学医院接受治疗的所有神经结节病患者。
年龄大于18岁且符合神经结节病协会共识小组神经结节病诊断标准的患者纳入本研究。评估治疗反应以及最后一次随访时的残疾程度。
我们确定了48例患者，其中3例被归类为确诊神经结节病，30例可能为神经结节病，15例可能疑似神经结节病。颅神经病变是最常见的表现（47.9%），其次是脊髓病（25%）。95.83%的患者有全身受累，纵隔淋巴结是最常见的部位。65.8%的患者临床症状改善，28.9%病情稳定，而5.26%病情恶化。50%的患者康复且无任何残留残疾，26.3%有轻度残留后遗症，23.7%有重度残留后遗症。
神经结节病是一种多样的疾病，临床表现、治疗反应和预后各不相同。颅神经病变是最常见的表现特征，预后良好，而脊髓病预后不良。除非有全身受累，否则脑膜和脑实质疾病难以准确诊断。在适当的临床情况下应临床怀疑神经结节病，应调查是否存在全身受累，并应尝试进行组织学确诊。
