Matthews Lucy, Kolind Shannon, Brazier Alix, Leite Maria Isabel, Brooks Jonathan, Traboulsee Anthony, Jenkinson Mark, Johansen-Berg Heidi, Palace Jacqueline
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, United Kingdom; Neurology Department, Oxford University Hospitals NHS Trust, Oxford, United Kingdom.
Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, Canada.
PLoS One. 2015 Sep 18;10(9):e0137715. doi: 10.1371/journal.pone.0137715. eCollection 2015.
Inflammatory demyelinating lesions of the central nervous system are a common feature of both neuromyelitis optica and multiple sclerosis. Despite this similarity, it is evident clinically that the accumulation of disability in patients with neuromyelitis optica is relapse related and that a progressive phase is very uncommon. This poses the question whether there is any pathological evidence of disease activity or neurodegeneration in neuromyelitis optica between relapses. To investigate this we conducted a longitudinal advanced MRI study of the brain and spinal cord in neuromyelitis optica patients, comparing to patients with multiple sclerosis and controls. We found both cross-sectional and longitudinal evidence of diffusely distributed neurodegenerative surrogates in the multiple sclerosis group (including thalamic atrophy, cervical cord atrophy and progressive widespread diffusion and myelin water imaging abnormalities in the normal appearing white matter) but not in those with neuromyelitis optica, where localised abnormalities in the optic radiations of those with severe visual impairment were noted. In addition, between relapses, there were no new silent brain lesions in the neuromyelitis optica group. These findings indicate that global central nervous system neurodegeneration is not a feature of neuromyelitis optica. The work also questions the theory that neurodegeneration in multiple sclerosis is a chronic sequela to prior inflammatory and demyelinating pathology, as this has not been found to be the case in neuromyelitis optica where the lesions are often more destructive.
中枢神经系统的炎性脱髓鞘病变是视神经脊髓炎和多发性硬化的共同特征。尽管存在这种相似性,但临床上明显的是,视神经脊髓炎患者的残疾累积与复发相关,且进展期非常罕见。这就提出了一个问题,即在复发间期,视神经脊髓炎是否存在疾病活动或神经退行性变的病理证据。为了对此进行研究,我们对一组视神经脊髓炎患者的脑和脊髓进行了一项纵向高级磁共振成像(MRI)研究,并与多发性硬化患者及对照组进行比较。我们发现,在多发性硬化组中存在弥漫性分布的神经退行性替代指标的横断面和纵向证据(包括丘脑萎缩、颈髓萎缩以及正常外观白质中广泛的扩散和髓鞘水成像异常),但在视神经脊髓炎患者中未发现此类情况,不过在严重视力损害患者的视辐射中发现了局部异常。此外,在复发间期,视神经脊髓炎组未出现新的无症状脑病变。这些发现表明,中枢神经系统整体神经退行性变并非视神经脊髓炎的特征。这项研究还对多发性硬化中神经退行性变是先前炎症和脱髓鞘病理的慢性后遗症这一理论提出了质疑,因为在视神经脊髓炎中并未发现这种情况,而视神经脊髓炎的病变往往更具破坏性。