Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, Vita-Salute San Raffaele University, Via Olgettina 60, 20132 Milan, Italy.
J Neurol. 2012 Apr;259(4):611-21. doi: 10.1007/s00415-011-6195-6. Epub 2011 Aug 4.
Primary progressive multiple sclerosis (PPMS) is characterized by a steady progression of irreversible disability from the onset of the disease. Although magnetic resonance imaging (MRI) is a valuable tool to quantify the disease burden in the brain and spinal cord of patients with MS, measures derived from conventional MRI, including T2-visible lesions, gadolinium-enhancing lesions and atrophy, are correlated only weakly with the clinical manifestations of PPMS. On the contrary, advanced MRI techniques are contributing significantly to the understanding of the mechanisms underlying the irreversible accumulation of disability in PPMS patients. Data from quantitative MRI studies suggest that the extent and topography of "diffuse" damage in different central nervous system (CNS) compartments (i.e. normal-appearing brain white matter and grey matter and the spinal cord) is associated with the severity of disability in PPMS and can predict subsequent medium-term disease evolution. Functional MRI studies have shown that the impairment of the adaptive capacity of the cortex to limit the clinical consequences of structural CNS damage is yet another factor contributing to the manifestations of this condition.
原发性进展型多发性硬化症（PPMS）的特征是从疾病发作开始，不可逆转的残疾逐渐加重。虽然磁共振成像（MRI）是一种评估 MS 患者脑和脊髓疾病负担的有效工具，但源于常规 MRI 的指标，包括 T2 可见病灶、钆增强病灶和萎缩，与 PPMS 的临床表现相关性较弱。相反，高级 MRI 技术对理解导致 PPMS 患者残疾不可逆转积累的机制有重要贡献。定量 MRI 研究的数据表明，不同中枢神经系统（CNS）区域（即正常外观的脑白质和灰质以及脊髓）内“弥漫性”损伤的程度和分布与 PPMS 残疾的严重程度相关，并可预测中期疾病进展。功能 MRI 研究表明，大脑皮层适应能力的损害限制了结构性 CNS 损伤的临床后果，这是导致该疾病表现的另一个因素。
