An Overview of Frontotemporal Lobar Degeneration

There is a brief albeit comprehensive open-access review of Frontotemporal Lobar Degeneration (FTLD) by Sieben and colleagues in Act Neuropathologica. The authors have assembled findings from the research literature and presented the results in a highly technical but succinct way. The clinical presentation of FTLD in this review is divided into

1. Behavioural variant FTD (or bvFTD)

2. Primary Progressive Aphasia (PPA). PPA is further subdivided according to recent consensus criteria into

  • Nonfluent/agrammatic variant PPA
  • Semantic variant PPA
  • Logopenic variant PPA

The authors also identify the main autosomal dominant genes associated with FTLD

  • MAPT
  • GRN
  • C9orf72
  • VCP
  • CHMP2B
  • TARDBP and FUS (more commonly associated with Amyotrophic Lateral Sclerosis than FTLD)

The authors expand on each gene and identify clinicopathological correlates although these are far from straightforward. There is also a brief section on susceptibility genes. The authors have produced a useful overview of FTLD which incorporates recent findings and elucidates the relationship of FTLD with a number of important gene associations. The review offers a useful starting point for further exploration of the literature.

Index: There are indices for the TAWOP site here and here Twitter: You can follow ‘The Amazing World of Psychiatry’ Twitter by clicking on this link. Podcast: You can listen to this post on Odiogo by clicking on this link (there may be a small delay between publishing of the blog article and the availability of the podcast). It is available for a limited period. TAWOP Channel: You can follow the TAWOP Channel on YouTube by clicking on this link. Responses: If you have any comments, you can leave them below or alternatively e-mail Disclaimer: The comments made here represent the opinions of the author and do not represent the profession or any body/organisation. The comments made here are not meant as a source of medical advice and those seeking medical advice are advised to consult with their own doctor. The author is not responsible for the contents of any external sites that are linked to in this blog.

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