Neurological complications of primary Sjogren's syndrome

Neurological complications of primary Sjogren's syndrome
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DOI:
10.1007/s004150170135
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发表时间:
2001-07-01
影响因子:
6
通讯作者:
Delattre, JY
Delattre, JY
中科院分区:
医学2区
文献类型:
--
作者:
Lafitte, C;Amoura, Z;Delattre, JY

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目的:更好地描述原发性干燥综合征(PSS)神经系统并发症谱。研究方法:对1996年6月至1997年12月在内科就诊的25例PSS患者进行了详细的神经系统前瞻性研究(内科组)。此外,在同一时期,在同一机构的神经科确定了11例PSS神经系统并发症患者(神经系统组)。结果:内科组25例患者中有10例(40%)出现神经系统并发症。内科组4/25例患者和神经组10/11例患者存在周围神经系统受累,主要包括轴突感觉运动/感觉性多发性神经病。在两名患者中发现了运动神经元综合征。内科组7/25例患者和神经组4/11例患者发生CNS受累。3例患者有脊髓受累。认知功能障碍是最常见的发现(内科组5/25,神经组3/11),其特征为皮质下或皮质下皮质功能障碍。认知障碍不归因于情绪障碍,也与特定的实验室或放射学异常无关。结论:PSS的神经系统并发症很常见,因为在内科的连续系列患者中,40%(10/25)的患者存在这些并发症。虽然PNS的参与占主导地位,并发症的PSS影响大脑或脊髓并不罕见,与皮质下功能障碍的主要发现。
Objective: To better delineate the spectrum of neurological complications of primary Sjogren's syndrome (PSS). Methods: A detailed neurological investigation was prospectively performed in a group of 25 consecutive patients with PSS followed in an internal medicine department between June 1996 and December 1997 (Internal Medicine group). In addition, eleven patients with neurological complications of PSS were identified in the Neurological Department of the same institution during the same period (Neurological group). Results: In the Internal Medicine group, neurological complications were discovered in 10/25 (40%) patients. Peripheral nervous system involvement was present in 4/25 patients from the Internal Medicine group and in 10/11 patients from the Neurological group and consisted mainly of axonal sensorimotor/sensory polyneuropathy. A motor neuron syndrome was identified in two patients. CNS involvement occurred in 7/25 patients from the Internal Medicine group and in 4/11 patients from the Neurological group. Three patients had spinal cord involvement. Cognitive dysfunction was the most frequent finding (5/25 in the Internal Medicine group, 3/11 in the Neurological group) characterized either by subcortical or corticosubcortical dysfunction. Cognitive impairment was not attributed to mood disturbance and was not associated with specific laboratory or radiological abnormalities. Conclusion: Neurological complications of PSS are frequent since they were present in 40% (10/25) of patients in a consecutive series of patients from a department of Internal Medicine. Although PNS involvement predominates, complications of PSS affecting the brain or spinal cord are not rare, with subcortical dysfunction as the main finding.