Poser C M, Brinar V V
Department of Neurology, Harvard Medical School, Boston, MA, USA.
Acta Neurol Scand. 2007 Oct;116(4):201-6. doi: 10.1111/j.1600-0404.2007.00902.x.
The practice of initiating immunomodulatory treatment immediately after a clinically isolated syndrome (CIS) suggestive of multiple sclerosis (MS) emphasizes the need to distinguish between disseminated encephalomyelitis (DEM) and MS. Their clinical, genetic, imaging, and histopathological characteristics establish that they are distinct disease entities. Acute and recurrent DEM are more common in children, but also occur in adults. DEM is polysymptomatic and includes signs and symptoms rarely encountered in MS, such as fever, alterations of the state of consciousness, cognitive and aphasic symptoms, and meningism. Cerebrospinal oligoclonal bands are rare. Magnetic resonance imaging (MRI) is the best means of distinguishing between DEM and MS. In the former, the lesion load is heavy, thalamus or basal ganglia are often affected, and early in the disease most of the lesions are usually larger than those of MS and enhance with gadolinium. The MRI spinal cord lesions are longer than three vertebral segments, and define neuromyelitis optica (NMO). Antibodies against aquaporin-4 are present in some NMO, but are also found in cases of MS and DEM. Most NMO are forms of DEM, not MS, and are identical with the 'Oriental' or 'optico-spinal' form of MS.
对于疑似多发性硬化症(MS)的临床孤立综合征(CIS)立即启动免疫调节治疗的做法,凸显了区分播散性脑脊髓炎(DEM)和MS的必要性。它们的临床、遗传、影像学和组织病理学特征表明,它们是不同的疾病实体。急性复发性DEM在儿童中更为常见,但也见于成人。DEM症状多样,包括一些在MS中很少出现的体征和症状,如发热、意识状态改变、认知和失语症状以及脑膜刺激征。脑脊液寡克隆带罕见。磁共振成像(MRI)是区分DEM和MS的最佳方法。在DEM中,病灶负荷较重,丘脑或基底节常受累,且在疾病早期,大多数病灶通常比MS的病灶大,并且钆增强。MRI脊髓病灶长度超过三个椎体节段,则可诊断为视神经脊髓炎(NMO)。部分NMO患者存在水通道蛋白4抗体,但在MS和DEM病例中也可检测到。大多数NMO属于DEM而非MS,等同于MS的“东方型”或“视神经脊髓型”。