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印度南部一家三级医疗中心的神经干燥综合征谱系

Spectrum of Neuro-Sjogren's syndrome in a tertiary care center in south India.

作者信息

Yadav Ravi, Krishnan Pramod, Kulkarni Girish Baburao, Yasha T C, Kumar M Veerendra, Nagraja D

机构信息

Department of Neurology, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, India.

出版信息

Ann Indian Acad Neurol. 2011 Apr;14(2):111-5. doi: 10.4103/0972-2327.82797.

DOI:10.4103/0972-2327.82797
PMID:21808473
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3141473/
Abstract

BACKGROUND

Neurological affection in Sjogren's syndrome (SS) can occur in the central and peripheral nervous system. Literature describing the neurological involvement in SS among Indian patients is lacking.

MATERIALS AND METHODS

Six patients of SS fulfilling the histological or serological criteria of the American European Consensus Group for SS were studied prospectively. The patients underwent clinical examination and laboratory investigations. Their clinical and investigation features are described.

RESULTS

The age of the patients ranged from 26 to 48 years, with a male to female ratio of 2:4. In our series, peripheral sensori-motor neuropathy and sensory ataxic neuropathy was seen in 3/6, mononeuritis multiplex in 2/6, cranial neuropathy in 2/6, autonomic neuropathy in 1/6, myelopathy in 4/6, optic neuropathy in 2/6, with presence of classical sicca features in 5/6 patients. Positive lip biopsy was seen in three, altitudinal field defect in one and positive Schirmer's test in five patients. Nerve conduction study abnormalities were seen in three and evidence of vasculitis was seen in nerve biopsy of one patient and chronic nonuniform axonopathy was seen in another. Antibody to Ro (SSA) or La (SSB) was positive in five patients.

CONCLUSIONS

SS involves different parts of the nervous system with varied presentations. Clinical suspicion and adequate laboratory testing helps to diagnose and manage this disorder that is relatively rare in Indian patients.

摘要

背景

干燥综合征(SS)中的神经病变可发生于中枢和周围神经系统。目前缺乏关于印度患者中SS神经受累情况的文献报道。

材料与方法

对6例符合美国-欧洲干燥综合征共识小组组织学或血清学标准的SS患者进行前瞻性研究。患者接受了临床检查和实验室检查。描述了他们的临床及检查特征。

结果

患者年龄在26至48岁之间,男女比例为2:4。在我们的系列研究中,3/6的患者出现周围感觉运动神经病和感觉性共济失调性神经病,2/6的患者出现多发性单神经病,2/6的患者出现颅神经病,1/6的患者出现自主神经病,4/6的患者出现脊髓病,2/6的患者出现视神经病变,5/6的患者有典型的干燥症状。3例唇活检阳性,1例有高度视野缺损,5例泪液分泌试验阳性。3例患者神经传导研究异常,1例患者神经活检有血管炎证据,另1例有慢性不均一性轴索性神经病。5例患者抗Ro(SSA)或La(SSB)抗体阳性。

结论

SS累及神经系统的不同部位,表现多样。临床怀疑并进行充分的实验室检查有助于诊断和处理这种在印度患者中相对罕见的疾病。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17ff/3141473/0e509be5026d/AIAN-14-111-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17ff/3141473/0e509be5026d/AIAN-14-111-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/17ff/3141473/0e509be5026d/AIAN-14-111-g001.jpg

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