Sugano M, Hirayama K, Saito T, Tsukamoto T, Yamamoto T
Department of Neurology, Fukushima Medical College.
Rinsho Shinkeigaku. 1992 Jun;32(6):621-5.
We reported an unusual case of multiple sclerosis (MS), clinical manifestations and neuroimagings of which were restricted to the frontal lobes and Gd-enhancing plaques on MRI later changed to necrotic lesions. A 36-year-old previously healthy man developed behavioral changes without any other neurological manifestations in a few weeks. Findings of magnetic resonance imaging (MRI) together with visual evoked potentials pointed to MS and corticosteroid pulse-dose therapy was initiated. Despite fairly uneventful, but rather delayed clinical recovery, repeated MRI disclosed rows of multiple, periventricular plaques localized to the bilateral frontal white matter and indicated the majority of lesions were not those of demyelination but necrosis. Frontal lobe syndrome is rarely encountered as a sole manifestation and rates only a few percent of MS cases in the literature. We speculated on the pathogenesis of necrotic plaques in this particular case; tumor necrosis factor, interleukin-1 and other cytokines that are known to be accompaniments of inflammatory cell infiltrates and activated glia in MS may ultimately destroy the CNS tissue, if the inflammation is not readily controlled. It is not certain, however, that what are determinants of demyelination and what drives further to the devastating tissue necrosis. Despite the uncertainty, this case indicates a necessity of immediate institution of adequate immunosuppressive therapy when neuroimaging study indicates active inflammation and openings of the blood-brain barrier in demyelinating plaques.
我们报告了一例罕见的多发性硬化症(MS)病例,其临床表现和神经影像学表现均局限于额叶,MRI上钆增强斑块后来转变为坏死性病变。一名36岁此前健康的男性在几周内出现行为改变,无任何其他神经系统表现。磁共振成像(MRI)结果及视觉诱发电位提示为MS,遂开始给予大剂量皮质类固醇脉冲疗法。尽管临床恢复过程较为平稳但相当延迟,重复MRI显示双侧额叶白质有一排排多发的脑室周围斑块,提示大多数病变并非脱髓鞘病变而是坏死性病变。额叶综合征很少作为唯一表现出现,在文献报道的MS病例中仅占百分之几。我们推测了该特殊病例中坏死斑块的发病机制;已知在MS中伴随炎性细胞浸润和活化胶质细胞的肿瘤坏死因子、白细胞介素-1及其他细胞因子,如果炎症不能得到及时控制,最终可能会破坏中枢神经系统组织。然而,脱髓鞘的决定因素是什么以及是什么导致进一步的毁灭性组织坏死尚不确定。尽管存在不确定性,但该病例表明,当神经影像学研究显示脱髓鞘斑块中有活动性炎症和血脑屏障开放时,有必要立即给予充分的免疫抑制治疗。