Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, Scientific Institute and University Vita-Salute San Raffaele, Milan, Italy.
Cortex. 2012 Apr;48(4):389-413. doi: 10.1016/j.cortex.2011.04.012. Epub 2011 May 1.
Frontotemporal lobar degeneration (FTLD) is a clinically and pathologically heterogeneous spectrum of disorders. In the last few years, neuroimaging has contributed to the phenotypic characterisation of these patients. Complementary to the clinical and neuropsychological evaluations, structural magnetic resonance imaging (MRI) and functional techniques provide important pieces of information for the diagnosis of FTLD. They also appear to be useful in distinguishing FTLD from patients with Alzheimer's disease (AD). Preliminary studies in pathologically proven cases suggested that distinct patterns of tissue loss could assist in predicting in vivo the pathological subtype. Recent years have also witnessed impressive advances in the development of novel imaging approaches. Diffusion tensor MRI and functional MRI have improved our understanding of the pathophysiology of the disease, and this should lead to the identification of additional useful markers of disease progression. This reviews discusses comprehensively the state-of-the-art of neuroimaging in the study of FTLD spectrum of disorders, and attempts to envisage which will be new neuroimaging biomarkers that could serve as surrogate measures of the underlying pathology. This will be central in the design of treatment trials of experimental drugs, which are likely to emerge in the near future, to target the pathological processes associated with this condition.
额颞叶变性(FTLD)是一种临床和病理表现多样的疾病。在过去的几年中,神经影像学为这些患者的表型特征提供了帮助。除了临床和神经心理学评估外,结构磁共振成像(MRI)和功能技术为 FTLD 的诊断提供了重要信息。它们似乎也有助于将 FTLD 与阿尔茨海默病(AD)患者区分开来。在经过病理证实的病例的初步研究中,发现不同的组织丢失模式可以帮助预测体内的病理亚型。近年来,新型成像方法的发展也取得了令人瞩目的进展。弥散张量 MRI 和功能 MRI 提高了我们对疾病病理生理学的理解,这应该会识别出更多疾病进展的有用标志物。本文全面讨论了神经影像学在 FTLD 疾病谱研究中的最新进展,并试图设想哪些新的神经影像学生物标志物可以作为潜在病理的替代指标。这对于设计针对这种疾病相关病理过程的实验药物治疗试验至关重要,这些药物可能在不久的将来出现。