The wide spectrum of clinical manifestations in Sjogren's syndrome-associated neuropathy
The wide spectrum of clinical manifestations in Sjogren's syndrome-associated neuropathy
复制标题
DOI：
10.1093/brain/awh605
复制
发表时间：
2005-11-01
期刊：
影响因子：
14.5
通讯作者：
Sobue, G
中科院分区：
文献类型：
--
作者：
Mori, K;Iijima, M;Sobue, G
我们评估了92例原发性干燥综合征相关神经病的临床病理特征(女性76例，男性16例，发病年龄54.7岁)。大多数患者(93%)在出现神经病变症状后被诊断为干燥综合征。根据主要的神经病变症状，我们将这些患者分为7种类型：感觉共济失调神经病36例，痛性感觉神经共济失调18例，多发性单神经病11例，多发性颅神经病5例，三叉神经病15例，自主神经病3例，神经根神经病4例。急性或亚急性起病在多发性单神经病和多发性颅神经病中更常见，而慢性进展在其他形式的神经病中占主导地位。没有实质性运动受累的感觉症状主要见于感觉共济失调、疼痛感觉、三叉神经和自主神经病变，尽管受影响的感觉方式和分布模式各不相同。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均可出现运动无力和肌肉萎缩。自主神经症状常见于各种形式的神经病。在感觉性共济失调、疼痛、三叉神经病和自主神经病中，瞳孔异常和直立性低血压尤其常见。感觉性共济失调、痛性神经病和自主神经病的MRI表现为体感诱发电位异常和脊髓后柱异常。腓肠神经活检标本(n=55)显示不同程度的轴突丢失。在感觉性共济失调神经病中观察到的主要是大纤维丢失，而在痛性感觉神经病中主要是小纤维丢失。血管炎和血管周围细胞侵犯在多发性单神经病中最常见，其次是感觉性共济失调神经病。1例感觉性共济失调神经病的尸检结果显示，与脊髓后根和后柱受累平行的大量感觉神经元严重丢失，交感神经元严重丢失。背神经节和交感神经节神经元丢失的程度与感觉和出汗损害的节段性分布相对应。神经干的背根和交感神经节、神经周围间隙和血管壁可见多灶性T细胞侵犯。皮质类固醇和静脉注射免疫球蛋白在不同的神经病变形式中的治疗反应不同。这些临床病理观察提示，感觉性共济失调、痛性或三叉神经病与神经节细胞病理性突起有关，而多发性单神经性和多发性颅神经病与血管突起的关系更为密切。
We assessed the clinicopathological features of 92 patients with primary Sjogren's syndrome-associated neuropathy (76 women, 16 men, 54.7 years, age at onset). The majority of patients (93%) were diagnosed with Sjogren's syndrome after neuropathic symptoms appeared. We classified these patients into seven forms of neuropathy: sensory ataxic neuropathy (n = 36), painful sensory neuropathy without sensory ataxia (n = 18), multiple mononeuropathy (n = 11), multiple cranial neuropathy (n = 5), trigeminal neuropathy (n = 15), autonomic neuropathy (n = 3) and radiculoneuropathy (n = 4), based on the predominant neuropathic symptoms. Acute or subacute onset was seen more frequently in multiple mononeuropathy and multiple cranial neuropathy, whereas chronic progression was predominant in other forms of neuropathy. Sensory symptoms without substantial motor involvement were seen predominantly in sensory ataxic, painful sensory, trigeminal and autonomic neuropathy, although the affected sensory modalities and distribution pattern varied. In contrast, motor weakness and muscle atrophy were observed in multiple mononeuropathy, multiple cranial neuropathy and radiculoneuropathy. Autonomic symptoms were often seen in all forms of neuropathy. Abnormal pupils and orthostatic hypotension were particularly frequent in sensory ataxic, painful, trigeminal and autonomic neuropathy. Unelicited somatosensory evoked potentials and spinal cord posterior column abnormalities in MRI were observed in sensory ataxic, painful and autonomic neuropathy. Sural nerve biopsy specimens (n = 55) revealed variable degrees of axon loss. Predominantly large fibre loss was observed in sensory ataxic neuropathy, whereas predominantly small fibre loss occurred in painful sensory neuropathy. Angiitis and perivascular cell invasion were seen most frequently in multiple mononeuropathy, followed by sensory ataxic neuropathy. The autopsy findings of one patient with sensory ataxic neuropathy showed severe large sensory neuron loss paralleling to dorsal root and posterior column involvement of the spinal cord, and severe sympathetic neuron loss. Degrees of neuron loss in the dorsal and sympathetic ganglion corresponded to segmental distribution of sensory and sweating impairment. Multifocal T-cell invasion was seen in the dorsal root and sympathetic ganglion, perineurial space and vessel walls in the nerve trunks. Differential therapeutic responses for corticosteroids and IVIg were seen among the neuropathic forms. These clinicopathological observations suggest that sensory ataxic, painful and perhaps trigeminal neuropathy are related to ganglioneuronopathic process, whereas multiple mononeuropathy and multiple cranial neuropathy would be more closely associated with vasculitic process.