Frontotemporal dementia and related disorders: deciphering the enigma

Ann Neurol. 2008 Jul;64(1):4-14. doi: 10.1002/ana.21426.


In the past century, particularly the last decade, there has been enormous progress in our understanding of frontotemporal dementia, a non-Alzheimer's type dementia. Large clinicopathological series have been published that have clearly demonstrated an overlap between the clinical syndromes subsumed under the term frontotemporal dementia and the progressive supranuclear palsy syndrome, corticobasal syndrome, and motor neuron disease. There have also been significant advancements in brain imaging, neuropathology, and molecular genetics that have led to different approaches to classification. Unfortunately, the field is complicated by a barrage of overlapping clinical syndromes and histopathological diagnoses that does not allow one to easily identify relations between individual clinical syndromic presentations and underlying neuropathology. This review deciphers this web of terminology and highlights consistent, and hence important, associations between individual clinical syndromes and neuropathology. These associations could ultimately allow the identification of appropriate patient phenotypes for future targeted treatments.

Publication types

  • Review

MeSH terms

  • Basal Ganglia Diseases / classification
  • Basal Ganglia Diseases / diagnosis
  • Basal Ganglia Diseases / physiopathology
  • Cerebral Cortex / pathology
  • Cerebral Cortex / physiopathology*
  • Dementia / classification
  • Dementia / diagnosis*
  • Dementia / physiopathology*
  • Dementia / therapy
  • Diagnosis, Differential
  • Disease Progression
  • Genetic Markers
  • Humans
  • Motor Neuron Disease / classification
  • Motor Neuron Disease / diagnosis
  • Motor Neuron Disease / physiopathology
  • Neurons / pathology*
  • Supranuclear Palsy, Progressive / classification
  • Supranuclear Palsy, Progressive / diagnosis
  • Supranuclear Palsy, Progressive / physiopathology


  • Genetic Markers