Neuroimaging findings in frontotemporal lobar degeneration spectrum of disorders

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.