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
- 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.
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