de Seze J, Stojkovic T, Hachulla E, Breteau G, Michon-Pasturel U, Mounier-Vehier F, Hatron P Y, Vermersch P
Service de Neurologie, Hôpital R. Salengro, CHRU de Lille, France.
Rev Neurol (Paris). 2001 Jul;157(6-7):669-78.
Myelopathies associated with Sjögren's syndrome has been rarely described especially concerning magnetic resonance imaging (MRI) and treatment aspects. The aim of this study was to determine the clinical, laboratory and radiological features of myelopathies occurring in Sjögren's syndrome. Eleven patients were studied, 7 with an acute myelopathy and 4 with a chronic form. Acute myelopathy were clinically severe with a feature of transverse myelitis necessitating immunosuppressive drugs. On the other hand, chronic forms were closely similar to progressive multiple sclerosis (MS), for clinical and laboratory data. In 7 cases optic neuritis was found associated with myelopathy and fulfilled the diagnostic criteria of Devic's syndrome in 4 cases. The diagnosis of myelopathy associated with Sjögren's syndrome may be difficult especially compared with MS, HTLV1 or HIV myelopathy and sarcoidosis, in the chronic form but also with other vasculitis, MS or viral infection in the acute forms. However, in this last form, magnetic resonance imaging and cerebrospinal fluid data should bring to the diagnosis of Sjögren syndrome and confirmed by appropriate tests. This diagnosis will have direct consequences for an early treatment by immunosuppressive drugs.
与干燥综合征相关的脊髓病鲜有报道，尤其是在磁共振成像（MRI）和治疗方面。本研究的目的是确定干燥综合征中发生的脊髓病的临床、实验室和放射学特征。对11例患者进行了研究，其中7例为急性脊髓病，4例为慢性脊髓病。急性脊髓病临床症状严重，具有横贯性脊髓炎的特征，需要使用免疫抑制药物。另一方面，慢性脊髓病在临床和实验室数据方面与进行性多发性硬化症（MS）非常相似。7例患者中发现视神经炎与脊髓病相关，4例符合视神经脊髓炎谱系疾病的诊断标准。与干燥综合征相关的脊髓病的诊断可能很困难，尤其是在慢性形式下，与MS、人类嗜T淋巴细胞病毒1型（HTLV1）或HIV脊髓病以及结节病相比，在急性形式下与其他血管炎、MS或病毒感染相比也是如此。然而，在急性形式下，磁共振成像和脑脊液数据应有助于干燥综合征的诊断，并通过适当的检查得到证实。这一诊断将对免疫抑制药物的早期治疗产生直接影响。
