Agraphia in patients with frontotemporal dementia and parkinsonism linked to chromosome 17 with P301L MAPT mutation: dysexecutive, aphasic, apraxic or spatial phenomenon?

Abstract

Objectives: Patients with frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) may be agraphic. The study aimed at characterizing agraphia in individuals with a P301L MAPT mutation.

Methods: Two pairs of siblings with FTDP-17 were longitudinally examined for agraphia in relation to language and cognitive deficits.

Results: All patients presented with dysexecutive agraphia. In addition, in the first pair of siblings one sibling demonstrated spatial agraphia with less pronounced allographic agraphia and the other sibling had aphasic agraphia. Aphasic agraphia was also present in one sibling from the second pair.

Conclusion: Agraphia associated with FTDP-17 is very heterogeneous.

Keywords:

• Frontotemporal dementia
• Writing
• Neuropsychological assessment
• Dysexecutive agraphia
• Spatial agraphia
• Aphasic agraphia

We wish to thank patients and all family members for their participation and interest in our study. EJS was supported by the START Scholarship awarded by the Foundation for the Polish Science. Both EJS and MH received a scholarship from the Polish Ministry of Science and Higher Education. BJM was supported by the Robert and Clarice Smith Fellowship Program, Mayo Clinic Florida, the Medical University of Silesia, Poland, and the Polish Foundation for Development of Neurology, Degenerative and Cerebrovascular Diseases. ZKW was supported by a Morris K. Udall NIH/NINDS Parkinson’s Disease Research Center of Excellence (P50NS072187), NIH/NINDS (1RC2NS070276 and NS057567), and Mayo Clinic Florida CR Program (MCF 90052018 and MCF 90052030) grants and by Carl Edward Bolch, Jr. and Susan Bass Bolch Gift Fund. RR was supported by the National Institute of Health (grant # NS65782).