Amyotrophic lateral sclerosis and frontotemporal dementia

Recent studies have shown a relation between ALS and frontotemporal dementia (FTD). The most common form of FTD, the behavioural variant, is characterized by disinhibition, apathy or executive dysfunction (planning, double-tasks). One in twelve patients develops FTD or has FTD at the time of ALS diagnosis.
The research team has developed a disease-specific questionnaire to detect behavioural changes in ALS patients. As one of the symptoms of FTD is lack of (disease)insight, the questionnaire has to be filled in by a partner or a family member of the patient.
The ALS-FTD-Q has been developed to be used by doctors and paramedics in order to gain insight into the extent of the behavioural changes. It can also be used for research purposes.

Use of the ALS-FTD-Q

The ALS-FTD-Q is currently being used in several countries, for clinical and research purposes. Validation studies of the English, Italian and Japanese version of the ALS-FTD-Q are in progress.

The Netherlands
Prof. M. de Visser, Academic Medical Centre, Amsterdam --> longitudinal study, responsivity ALS-FTD-Q
Prof. L. van den Berg, University Medical Center Utrecht, Utrecht --> case-control study
Prof. J. van Swieten, Erasmus Medical Center, Rotterdam --> divergent validity ALS-FTD-Q
Prof. P. Scheltens and dr. Y. Pijnenburg, VU medical center, Amsterdam --> clinical use

Prof. A. Chìo, L'università di Torino, Turin --> validation Italian version ALS-FTD-Q

Prof. K. Nakashima and dr. Y. Watanabe, Tottori University, Yonago --> validation of Japanese version ALS-FTD-Q

United States of America
Dr. R. Winnett, Virginia Mason Medical Center, Seattle --> pilot study English ALS-FTD-Q

Dr. L. Lacomblez, Groupe hospitalier Pitié-Salpêtrière, Paris --> prospective cohort study

Dr. E. Mioshi, Neuroscience Research Australia, Randwick NSW --> validation of English version ALS-FTD-Q

Prof. Z. Stević and dr. I. Marjanovic, Klinika za Neurologiju, Belgrade --> validation of Serbian version ALS-FTD-Q

Dr. O. Gredal, Rehabilitation Centre for Neuromuscular Diseases, Copenhagen --> prospective cohort study

Research team

Contact information

E. Beeldman and J. Raaphorst
Academic Medical Centre
Neurology department, room H2-235
PO box 22660
1100 DD Amsterdam
General inquiries? Click here.


1. Renton AE, Majounie E, Waite A, et al. A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Neuron 2011;72:257-268
2. Neumann M, Sampathu DM, Kwong LK, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science 2006;314:130-133
3. Raaphorst J, Beeldman E, De Visser M, De Haan RJ, Schmand B. A systematic review of behavioural changes in motor neuron disease. Amyotroph Lateral Scler 2012 Oct;13(6):493-501.


© All rights reserved, 2012. Academic Medical Centre (part of the ALS centre the Netherlands), in collaboration with the Alzheimer Centre of the VUmc, Amsterdam and the Erasmus MC, Rotterdam.