Koike Haruki, Sobue Gen
Department of Neurology, Nagoya University Graduate School of Medicine.
Brain Nerve. 2013 Nov;65(11):1333-42.
Sjogren's syndrome is a systemic autoimmune disease characterized by xerophthalmia and xerostomia; it is associated with widespread systemic visceral involvement. A wide variety of neurological complications are characteristic features of Sjogren's syndrome, of which peripheral neuropathy is a major neurological manifestation. Based on the predominant neuropathic symptoms, patients can be considered to have several forms of neuropathies, including sensory ataxic neuropathy, painful sensory neuropathy without sensory ataxia, multiple mononeuropathy, multiple cranial neuropathy, trigeminal neuropathy, autonomic neuropathy, and radiculoneuropathy. Acute or subacute onset is observed more frequently in multiple mononeuropathy and multiple cranial neuropathies, whereas disease progression is usually chronic in other forms of neuropathies. Sensory symptoms without substantial motor involvement are observed predominantly in sensory ataxic, painful sensory, trigeminal, and autonomic neuropathies. In contrast, motor impairment is apparent in multiple mononeuropathy, multiple cranial neuropathy, and radiculoneuropathy. Autonomic symptoms such as abnormal pupils and orthostatic hypotension are particularly noted in patients with sensory ataxic, painful, trigeminal, and autonomic neuropathies. Sural nerve biopsy specimens reveal predominantly large fiber loss in sensory ataxic neuropathy and predominantly small fiber loss in painful sensory neuropathy. Vasculitis is observed most frequently in multiple mononeuropathy. The autopsy findings of patients with sensory ataxic and painful neuropathies demonstrate neuronal loss in the dorsal root ganglia and sympathetic ganglia with CD8-positive cytotoxic T lymphocytes. Differential therapeutic responses to corticosteroids and intravenous immunoglobulin can be seen among the various neuropathic forms. In conclusion, the clinicopathological features of neuropathies associated with Sjogren's syndrome are highly variable. The neuropathy classification is important from a therapeutic point of view.
干燥综合征是一种以干眼症和口干症为特征的全身性自身免疫性疾病；它与广泛的全身内脏受累有关。多种神经并发症是干燥综合征的特征性表现，其中周围神经病变是主要的神经表现。根据主要的神经病变症状，患者可被认为患有多种形式的神经病变，包括感觉性共济失调性神经病变、无感觉性共济失调的疼痛性感觉神经病变、多发性单神经病、多发性颅神经病、三叉神经病、自主神经病变和神经根神经病。多发性单神经病和多发性颅神经病更常表现为急性或亚急性起病，而其他形式的神经病变疾病进展通常为慢性。感觉性共济失调性、疼痛性感觉、三叉神经和自主神经病变主要表现为无明显运动受累的感觉症状。相比之下，多发性单神经病、多发性颅神经病和神经根神经病中运动障碍明显。感觉性共济失调性、疼痛性、三叉神经和自主神经病变患者尤其会出现瞳孔异常和体位性低血压等自主神经症状。腓肠神经活检标本显示，感觉性共济失调性神经病变主要为大纤维丢失，疼痛性感觉神经病变主要为小纤维丢失。血管炎最常出现在多发性单神经病中。感觉性共济失调性和疼痛性神经病变患者的尸检结果显示，背根神经节和交感神经节有神经元丢失，伴有CD8阳性细胞毒性T淋巴细胞。不同类型的神经病变对皮质类固醇和静脉注射免疫球蛋白的治疗反应不同。总之，干燥综合征相关神经病变的临床病理特征高度可变。从治疗角度来看，神经病变的分类很重要。
