• DOI:10.1055/s-0039-1683379
• Corpus ID: 88481297

Frontotemporal Dementia: A Clinical Review.

@article{Sivasathiaseelan2019FrontotemporalDA,
  title={Frontotemporal Dementia: A Clinical Review.},
  author={Harri Sivasathiaseelan and Charles R. Marshall and Jennifer L. Agustus and Elia Benhamou and Rebecca L. Bond and Janneke E P van Leeuwen and Chris JD. Hardy and Jonathan Daniel Rohrer and Jason D. Warren},
  journal={Seminars in neurology},
  year={2019},
  volume={39 2},
  pages={
          251-263
        }
}

• H. Sivasathiaseelan, C. Marshall, +6 authors J. Warren
• Published 29 March 2019
• Medicine, Biology, Psychology
• Seminars in neurology

Frontotemporal dementias are a clinically, neuroanatomically, and pathologically diverse group of diseases that collectively constitute an important cause of young-onset dementia. Clinically, frontotemporal dementias characteristically strike capacities that define us as individuals, presenting broadly as disorders of social behavior or language. Neurobiologically, these diseases can be regarded as "molecular nexopathies," a paradigm for selective targeting and destruction of brain networks by pathogenic proteins. Mutations in three major genes collectively account for a substantial proportion of behavioral presentations, with far-reaching implications for the lives of families but also potential opportunities for presymptomatic diagnosis and intervention. Predicting molecular pathology from clinical and radiological phenotypes remains challenging; however, certain patterns have been identified, and genetically mediated forms of frontotemporal dementia have spearheaded this enterprise. Here we present a clinical roadmap for diagnosis and assessment of the frontotemporal dementias, motivated by our emerging understanding of the mechanisms by which pathogenic protein effects at the cellular level translate to abnormal neural network physiology and ultimately, complex clinical symptoms. We conclude by outlining principles of management and prospects for disease modification.