Primary progressive aphasia (PPA) is a rare clinical dementia syndrome affecting predominantly language abilities. Word-finding difficulties and comprehension deficits despite relatively preserved cognitive functions are characteristic symptoms during the first two years, and distinguish PPA from other dementia types like Alzheimer's disease. However, the dynamics of changes in language and non-linguistic abilities are not well understood. Most studies on progression used cross-sectional designs, which provide only limited insight into the course of the disease. Here we report the results of a longitudinal study in three cases of logopenic PPA over a period of 18 months, with exemplary longitudinal data from one patient even over 46 months. A comprehensive battery of neurolinguistic and neuropsychological tests was applied four times at intervals of six months. Over this period, deterioration of verbal abilities such as picture naming, story retelling, and semantic word recall was found, and the individual decline was quantified and compared between the three patients. Furthermore, decrease in non-verbal skills such as divided attention and increasing apraxia was observed in all three patients. In addition, inter-subject variability in the progression with different focuses was observed, with one patient developing a non-fluent PPA variant. The longitudinal, multivariate investigation of logopenic PPA thus provides novel insights into the progressive deterioration of verbal as well as non-verbal abilities. These deficits may further interact and thus form a multi-causal basis for the patients' problems in every-day life which need to be considered when planning individually targeted intervention in PPA.
|Original language||American English|
|Number of pages||11|
|State||Published - Jun 2012|
Bibliographical noteFunding Information:
We are indebted to our patients AT, AS, and MW, who took the repeated efforts to travel the long way to Jülich for examination. We wish to thank Karl-Josef Langen and his team from the neurological research ward at the Research Centre Jülich. We also thank Klaus Willmes and Bruno Fimm for support with single-subject statistical analyses. This project was supported by a JARA – Translational Brain Medicine grant to MG and SH. The project “Helmholtz Alliance for Mental Health in an Ageing Society” (HelMA, HA-215) was supported within the framework of the “Helmholtz Impuls- und Vernetzungsfonds”.
- Fronto-temporal degeneration