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Abstract
Objectives Frontotemporal dementia (FTD) is a heterogeneous clinical syndrome linked to diverse types of underlying neuropathology. Diagnosis is mainly based on clinical presentation and accurate prediction of underlying neuropathology remains difficult.
Methods We present a large cohort of patients with FTD spectrum diseases (n=84). All patients were thoroughly characterised by cerebrospinal fluid (CSF) Alzheimer’s disease (AD) biomarkers, neuroimaging, neuropsychological testing and standardised apraxia screening.
Results A potential AD pathology was found in 43% of patients with FTD. CSF AD biomarker levels positively correlated with AD-typical apraxia scores in patients with FTD. The discriminative power of apraxia test results indicative of AD pathology was high (sensitivity: 90%, specificity: 66%).
Conclusions Apraxia is common in neurodegenerative dementias but under-represented in clinical workup and diagnostic criteria. Standardised apraxia screening may serve as bedside test to objectify an AD-typical apraxia profile as an early and robust sign of AD pathology in patients with FTD.
- Alzheimer’s disease
- frontotemporal dementia
- primary progressive aphasia
- apraxia
- biomarker
- amyloid pathology
- default mode network
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Footnotes
MP, TD and AJ contributed equally.
Contributors MP performed statistical analyses, created figures and tables, wrote and revised the manuscript. VJ screened clinical records and created first drafts of figures and tables. HW and SGM helped design the study and reviewed the manuscript for medical content. TD helped design and supervised the study, recruited and treated patients. AJ designed the study, conducted neuropsychological assessments, performed statistical analyses, supervised draft and revision of the manuscript.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. MP, VJ and AJ have nothing to disclose. HW receives honoraria for acting as a member of Scientific Advisory Boards and as consultant for Biogen, Evgen, MedDay Pharmaceuticals, Merck Serono, Novartis, Roche Pharma and Sanofi-Genzyme, as well as speaker honoraria and travel support from Alexion, Biogen, Cognomed, F Hoffmann-La Roche, Gemeinnützige Hertie-Stiftung, Merck Serono, Novartis, Roche Pharma, Sanofi- Genzyme, TEVA and WebMD Global. HW is acting as a paid consultant for AbbVie, Actelion, Biogen, IGES, Novartis, Roche, Sanofi-Genzyme and the Swiss Multiple Sclerosis Society. His research is funded by the German Ministry for Education and Research (BMBF), Deutsche Forschungsgesellschaft (DFG), Else Kröner Fresenius Foundation, Fresenius Foundation, Hertie Foundation, NRW Ministry of Education and Research, Interdisciplinary Center for Clinical Studies (IZKF) Muenster and RE Children’s Foundation, Biogen, GlaxoSmithKline, Roche Pharma and Sanofi-Genzyme. SGM has received honoraria for lecturing, travel expenses for attending meetings and financial research support from Almirall, Bayer Health Care, Biogen, Diamed, Fresenius Medical Care, Genzyme, Merck Serono, Novartis, Novo Nordisk, ONO Pharma, Roche, Sanofi-Aventis and Teva. TD has received speaker honoraria, consultancy fees and travel expenses from Genzyme, Shire, Sanofi Aventis, Novartis, Actelion Pharmaceuticals and Amicus, research support from Genzyme, Shire, Amicus and Actelion Pharmaceuticals, and educational grants from Novartis, Roche and Biogen.
Patient consent Obtained.
Ethics approval Local ethics committee.
Provenance and peer review Not commissioned; externally peer reviewed.