Karaca Sibel, Ersözlü Bozkirli Emine Duygu, Karakurum Göksel Başak, Tan Meliha, Yücel Ahmet Eftal
Başkent University Faculty of Medicine, Adana Research and Application Center, Department of Neurology, Adana, Turkey.
Başkent University Faculty of Medicine, Adana Research and Application Center, Department of Rheumatology, Adana, Turkey.
Noro Psikiyatr Ars. 2014 Jun;51(2):148-156. doi: 10.4274/npa.y6911. Epub 2014 Jun 1.
Neurological involvements were shown in 20% of patients with Primary Sjogren's Syndrome (pSS). Neurological symptoms may be the first signs of pSS in 57% of the cases. In addition, early diagnosis and treatment of neurological disorders may save or improve the quality of life of these cases. There have been reports about the neurologic manifestations of pSS but little is known about the details of neurologically presented cases.
In this study, we described 11 pSS patients who presented with neurological manifestations.
Central nervous system (CNS) involvement was recorded in 7 (63.7%) and peripheric nervous system (PNS) involvement in 4 cases (36.4%).
Our findings regarding the cases with neurological manifestations leading to the diagnosis of pSS suggest that: 1) The frequency of CNS involvement was higher than that of PNS, and the most frequent clinical pictures of CNS involvement are Multiple Sclerosis (MS)-like illnesses and optic neuritis, 2) Guillain Barre Syndrome (GBS) was the most frequent disease of PNS involvement; 3) Mononeuropathy multiplex (MM) might be the first sign of pSS; 4) Neurologists should consider pSS in the differential diagnosis of cases with MS, optic neuritis, GBS and neuropathies of unknown causes including MM; 5) There is an urgent need of therapeutical guidelines for the cases with neurological involvement associated with pSS.
原发性干燥综合征(pSS)患者中有20%出现神经受累情况。在57%的病例中,神经症状可能是pSS的首发症状。此外,早期诊断和治疗神经疾病可能挽救或改善这些病例的生活质量。已有关于pSS神经表现的报道,但对于以神经症状就诊病例的详细情况知之甚少。
在本研究中,我们描述了11例出现神经表现的pSS患者。
记录到7例(63.7%)有中枢神经系统(CNS)受累,4例(36.4%)有外周神经系统(PNS)受累。
我们对因神经表现而诊断为pSS病例的研究结果表明:1)CNS受累的频率高于PNS,CNS受累最常见的临床表现是类多发性硬化(MS)疾病和视神经炎;2)格林-巴利综合征(GBS)是PNS受累最常见的疾病;3)多灶性单神经病(MM)可能是pSS的首发症状;4)神经科医生在对MS、视神经炎、GBS以及包括MM在内病因不明的神经病病例进行鉴别诊断时应考虑pSS;5)迫切需要针对与pSS相关神经受累病例的治疗指南。