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

Neurocase. 2014;20(1):69-86. doi: 10.1080/13554794.2012.732087. Epub 2012 Nov 5.

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.

Publication types

  • Case Reports
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Agraphia / diagnosis*
  • Agraphia / genetics*
  • Brain / pathology
  • Chromosomes, Human, Pair 17*
  • Disease Progression
  • Female
  • Frontotemporal Dementia / genetics*
  • Frontotemporal Dementia / pathology
  • Frontotemporal Dementia / psychology
  • Humans
  • Longitudinal Studies
  • Male
  • Middle Aged
  • Mutation
  • Parkinsonian Disorders / genetics*
  • Parkinsonian Disorders / pathology
  • Parkinsonian Disorders / psychology
  • tau Proteins / genetics*

Substances

  • MAPT protein, human
  • tau Proteins