Pou Serradell A, Roquer González J, Perich Alsina X
Service de Neurologie, Hospital Universitari del Mar, Universitat Autonoma de Barcelona, Barcelona.
Rev Neurol (Paris). 2000 Dec;156(12):1126-35.
Twenty patients with multiple sclerosis (MS), 19 women and 1 man, with acute proprioceptive sensory disturbances related to the presence of plaques on the posterior columns (posterior column syndrome) at the cervical or thoracic levels of the spinal cord, were selected among 138 new patients with MS assisted in our neurological unit over the past five years. In 17 of these patients, the acute posterior cordonal syndrome was responsible for the first clinical manifestations of the disease. The other 3 patients had a history suggestive of MS. These 20 patients were followed with a minute analysis of neurological function with repeated clinical evaluation combined with repeated MRI study of the spinal cord. Brain MRI (strongly suggestive of MS in 15 patients), evoked potentials (EP) and cerebrospinal fluid electrophoresis analysis (with oligoclonal bands present in all patients were it was performed) were also obtained at least once in each patient. Spinal cord MRI demonstrated more lesions in the cervical region (90 p.100) than in the thoracic regions (10 p.100). Eighty percent of the cervical lesions were located high, between C1 and C4. The most characteristic clinical expression was the deafferentation of one upper limb, preferentially the "useless hand" (Oppenheim) or even a pseudoathetosic or dystonic limb. Propioceptive ataxia or spontaneous cervical or brachial pain were other forms of clinical expression. No major motor deficit or sphincter disorders were noted at any time in the clinical course in any of the patients. There was a good correlation between localization and morphology of the plaques detected by spinal cord MRI and clinical signs. Intrinsic medullary lesions were seen as high intensity signals on T2-weighted images which were enlarged more than the same lesion visualized on T1-weighted images after injection of paramagnetic contrast agents. This reflected the presence of edema extending beyond the main inflammatory lesion. There was also a good correlation between improvement of clinical symptoms and total or, mor frequently, partial reduction of the plaques, analyzed morphologically by successive spinal cord MRI series. The diagnosis of MS was clinically definitive in 60 p.100 of cases and laboratory-supported definitive in 40 p.100. During the follow-up period (average 36 months), 15 patients (75 p.100) presented one or more exacerbations, all of them presenting a favorable course: at last follow-up, 9 patients were asymptomatic, EDSS was 1 in 6 patients, 1.5 in 4 patients and 2 in 1 patient. This study confirms the contribution of serial spinal cord MR studies to understanding the natural history and pathophysiology of medullary forms of MS presenting as a cordonal posterior syndrome. It also shows a good relationship between the clinical manifestations and course of this form of MS and the localization and variable morphology of plaques. Finally, our results suggest the predictive benign course for this medullary form of MS that seems to be almost exclusively restricted to the female gender.
在过去五年里,我们神经科收治的138例新发多发性硬化症(MS)患者中,挑选出20例患者,其中19名女性,1名男性。这些患者存在急性本体感觉障碍,与脊髓颈段或胸段后柱(后柱综合征)出现斑块有关。在这20例患者中,17例患者的急性后索综合征是该疾病的首发临床表现。另外3例患者有提示MS的病史。对这20例患者进行了细致的神经功能分析,通过反复的临床评估以及脊髓的重复MRI研究。每位患者至少进行过一次脑部MRI(15例患者强烈提示MS)、诱发电位(EP)以及脑脊液电泳分析(所有进行该项检查的患者均发现寡克隆带)。脊髓MRI显示,颈段病变(占90%)多于胸段病变(占10%)。80%的颈段病变位于高位,即C1至C4之间。最具特征性的临床表现是一侧上肢的传入神经阻滞,优先出现“无用手”(奥本海姆征),甚至出现假性手足徐动或张力障碍性肢体。本体感觉性共济失调或自发性颈部或臂部疼痛是其他临床表现形式。在任何患者的临床病程中,均未发现严重运动功能缺损或括约肌功能障碍。脊髓MRI检测到的斑块定位与形态和临床体征之间存在良好的相关性。脊髓内病变在T2加权图像上表现为高强度信号,注射顺磁性造影剂后,这些病变在T1加权图像上的扩大程度超过相同病变。这反映出水肿超出了主要炎症病变的范围。临床症状的改善与通过连续脊髓MRI系列进行形态学分析的斑块完全或更常见的部分缩小之间也存在良好的相关性。60%的病例临床诊断为MS,40%的病例有实验室支持的确诊。在随访期(平均3年)内,15例患者(占75%)出现一次或多次病情加重,但所有患者病情均呈良性转归:在最后一次随访时,9例患者无症状,6例患者的扩展残疾状态量表(EDSS)评分为1,4例患者为1.5,1例患者为2。这项研究证实了连续脊髓MR研究有助于理解表现为后索综合征的MS脊髓型的自然病史和病理生理学。它还显示了这种MS类型的临床表现和病程与斑块定位及形态变化之间的良好关系。最后,我们的结果提示这种MS脊髓型可能具有良性的病程预测,且似乎几乎仅见于女性。