Andonopoulos A P, Lagos G, Drosos A A, Moutsopoulos H M
Department of Medicine, Ioannina University School of Medicine, Greece.
J Autoimmun. 1989 Aug;2(4):485-8. doi: 10.1016/0896-8411(89)90179-0.
Forty consecutive patients with primary Sjögren's syndrome (SS) were prospectively evaluated for evidence of neurologic manifestations of the disease. All patients had primary SS diagnosed on the basis of typical sicca symptomatology, objective documentation of keratoconjunctivitis sicca (KCS) and/or xerostomia, and a positive minor labial salivary gland biopsy. None had criteria that would classify him/her as systemic lupus. Evaluation included a detailed neurologic history and physical examination and nerve-conduction studies of all four extremities. Ten patients had a mild sensory or mixed neuropathy of the glove-stocking pattern, and six of them had abnormal nerve conduction studies. Two of those, plus one more, had trigeminal neuropathy. Two more patients had abnormal electrophysiologic studies, one of them with absence of Achilles tendon reflexes as well. None of the above patients volunteered complaints related to these findings. Only one patient with severe primary SS, manifested by cryoglobulinemia, vasculitis and glomerulonephritis, presented with mononeuritis multiplex, that partially responded to intravenous cyclophosphamide and high-dose steroids. Central nervous system (CNS) disease was not detected in any of our patients, suggesting that it is rather rare, whereas peripheral nervous system (PNS) involvement is relatively common and benign in the majority of primary SS individuals.
对40例原发性干燥综合征（SS）患者进行前瞻性评估，以寻找该疾病神经学表现的证据。所有患者均根据典型的干燥症状、干燥性角结膜炎（KCS）和/或口干症的客观记录以及唇小唾液腺活检阳性确诊为原发性SS。没有人有将其归类为系统性红斑狼疮的标准。评估包括详细的神经病史、体格检查以及对四肢进行神经传导研究。10例患者有轻度的手套-袜套样感觉或混合性神经病变，其中6例神经传导研究异常。其中2例，另外还有1例有三叉神经病变。另外2例患者电生理研究异常，其中1例还存在跟腱反射消失。上述患者均未主动诉说与这些发现相关的症状。只有1例患有严重原发性SS（表现为冷球蛋白血症、血管炎和肾小球肾炎）的患者出现了多发性单神经炎，对静脉注射环磷酰胺和大剂量类固醇治疗有部分反应。在我们所有患者中均未检测到中枢神经系统（CNS）疾病，提示其相当罕见，而在大多数原发性SS患者中，周围神经系统（PNS）受累相对常见且为良性。
