Hagiwara Koichi, Murai Hiroyuki, Ochi Hirofumi, Osoegawa Manabu, Shigeto Hiroshi, Ohyagi Yasumasa, Kira Jun-Ichi
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka.
Intern Med. 2008;47(11):1047-51. doi: 10.2169/internalmedicine.47.0846. Epub 2008 Jun 2.
We present two patients with primary lateral sclerosis-like upper motor neuron disease accompanying subclinical Sjögren's syndrome. Both patients showed progressive spastic quadriparesis, but neither sensory involvement nor detrusor dysfunction was noted. Lower motor neuron signs were detected only in their late follow-up period. Although sicca symptom was nearly absent, salivary labial gland biopsy revealed marked sialoadenitis in both patients. They also displayed a constellation of findings that suggested an autoimmune etiology closely related to Sjögren's syndrome, including germinal center formation in one patient, and markedly elevated levels of anti-nuclear antibody with abnormal sialography in the other. Both patients showed significant neurological improvement after the initial course of intravenous immunoglobulin therapy. We suggest that the evidence for subclinical Sjögren's syndrome should be sought in patients presenting with selective upper motor neuron involvement.
我们报告了两名患有原发性侧索硬化样上运动神经元疾病并伴有亚临床干燥综合征的患者。两名患者均表现为进行性痉挛性四肢瘫，但均未发现感觉障碍或逼尿肌功能障碍。仅在随访后期检测到下运动神经元体征。尽管几乎没有口干症状，但唇唾液腺活检显示两名患者均有明显的涎腺炎。他们还表现出一系列提示与干燥综合征密切相关的自身免疫病因的发现，包括一名患者有生发中心形成，另一名患者抗核抗体水平显著升高且唾液造影异常。两名患者在初始静脉注射免疫球蛋白治疗疗程后均显示出明显的神经功能改善。我们建议，对于表现为选择性上运动神经元受累的患者，应寻找亚临床干燥综合征的证据。
