干燥综合征神经系统损害临床表现及诊治

Clinical manifestation, diagnosis and treatment of neurologic impairment of Sj?gren's syndrome

干燥综合征(Sj?gren’s syndrome,SS)是一种以口眼干燥、特异性自身抗体为特征的系统性自身免疫性疾病。约20%的SS患者伴有神经系统损害,表现为外周神经系统(peripheral nervous system,PNS)损害和中枢神经系统(central nervous system,CNS)损害。PNS损害包括感觉共济失调、单纯痛觉神经病、感觉神经节神经病、多发颅神经病、三叉神经病、多发单神经病、自律神经病和神经根病;CNS损害主要表现为头痛、脊髓炎、视神经脊髓炎、癫痫、中枢性脱髓鞘样改变、无菌性脑膜炎和认识障碍。目前,SS患者伴发神经系统损害的标准治疗仍为糖皮质激素联合免疫抑制剂。

Sjogren＇s Syndrome （SS） is an autoimmune disease characterized by sicca symptoms, anti-SS-A （Ro） or anti-SS-B （La） autoantibodies. Increasing evidence indicates that nervous system is involved in 20% patients with SS. Nervous impairments include peripheral neuropathies and central neuropathies. Peripheral neuropathies associated with SS include sensory ataxic neuropathy, painful sensory neuropathy without sensory ataxia, ganglionopathies, multiple mononeuropathy, cranial neuropathy3 trigeminal neuropathy, autonomic neuropathy, and radiculoneuropathy. CNS disorders include headache, spinal cord involvement, neuromyelitis optica, seizures, motor and sensory deficit, movement disorders, and aseptic meningitis. To date, corticosteroids and other immunosuppresjsive agents remain to be the primary treatment in the neuropathy associated with SS.