Escudero D, Latorre P, Codina M, Coll-Cantí J, Coll J
Department of Neurology, Hospital Universitari Germans Trias i Pujol, Badalona.
Ann Med Interne (Paris). 1995;146(4):239-42.
Our prospective study consisted of a general and neurological evaluation in 48 patients (41 females, 7 males; mean age: 58.2 years) with primary Sjögren's syndrome (PSS). We performed serologic studies and cranial magnetic resonance imaging (MRI). Main extraglandular features were arthralgias and non-erosive arthritis (37.5%). Raynaud (21%) and pulmonary fibrosis (12.5%). Antinuclear antibodies were positive in 42.5% and anti-SS-A (Ro) in 20%. Migraine (52%), neuropsychiatric disease (29%) and a past history of focal acute neurological deficits (23%), were the central nervous system (CNS) manifestations more frequently observed. Cranial MRI examination detected hyperintense small subcortical lesions in 51.3% of patients and in 36.6% of age and sex matched controls (P < 0.001). CNS disease was not serious concerning vital prognosis, but produced significant morbidity in some patients. Late onset "migraine-like" episodes with prolonged sensoromotor deficits and coexisting neuropsychiatric disease emerged as a characteristic clinical spectrum in those patients diagnosed in a neurological setting. Cranial MRI was frequently abnormal, but findings were not specific. Neurologic manifestations reminiscent of multiple sclerosis were rarely seen.
我们的前瞻性研究包括对48例原发性干燥综合征（PSS）患者（41例女性，7例男性；平均年龄：58.2岁）进行全面的体格检查和神经学评估。我们进行了血清学研究和头颅磁共振成像（MRI）检查。主要的腺外表现为关节痛和非侵蚀性关节炎（37.5%）、雷诺现象（21%）和肺纤维化（12.5%）。42.5%的患者抗核抗体呈阳性，20%的患者抗SS - A（Ro）呈阳性。偏头痛（52%）、神经精神疾病（29%）以及既往有局灶性急性神经功能缺损病史（23%）是最常观察到的中枢神经系统（CNS）表现。头颅MRI检查在51.3%的患者中检测到皮质下小的高信号病变，在36.6%年龄和性别匹配的对照者中也检测到（P < 0.001）。CNS疾病对生命预后而言并不严重，但在一些患者中导致了显著的发病情况。在神经科确诊的患者中，出现伴有延长的感觉运动功能缺损和并存神经精神疾病的迟发性“偏头痛样”发作，成为一种特征性的临床谱。头颅MRI经常显示异常，但结果并不具有特异性。很少见到类似多发性硬化的神经学表现。
