What dementia can do to language processing

The thesis at hand focuses on two main aspects: Firstly, the written text production in the preclinical phase of the semantic variant of primary progressive aphasia embedded in a longitudinal study; secondly, on the examination of Proper Names in patients suffering from Alzheimer´s Disease in a fluency task setting. Both studies focus on aspects which have not been subject to scientific research so far. The broad analysis of linguistics in written text production covered the levels of semantics, syntax and morphology and also included the examination of vocabulary and punctuation. The results showed an early onset of symptoms (approximately nine years before the clinical diagnose took place). Most surprising was the finding of an overwhelming amount of morphologic errors and an extreme usage of quotation marks. The results thus deepen the insight in the process of how the syndrome manifests itself in text production even before clinical diagnosis takes place. The fluency tasks for Proper Names proved to be promising for future usage as a screening tool for dementia. It could be shown that Proper Name fluency must be distinguished from Common Name fluency and that performance is different in patients suffering from AD and healthy elderly. Also, performance differs depending on the Proper Name fluency task.Die vorliegende Arbeit konzentriert sich auf zwei Schwerpunkte: Einerseits die schriftliche Textproduktion in der präklinischen Phase bei der semantischen Variante der Primär Progredienten Aphasie im Langzeitverlauf und andererseits die Untersuchung von Eigennamen bei Patienten mit Alzheimer Demenz in Form von fluency tasks. Beide Studien beleuchten Aspekte, zu denen bisher noch kaum wissenschaftliche Erkenntnisse vorliegen. In der umfassenden Studie zur schriftlichen Textproduktion wurden Aspekte aus den Bereichen Semantik, Syntax und Morphologie, sowie Vokabular und Zeichensetzung untersucht. Die Ergbnisse zeigen vor allem einen frühen Onset von ersten Symptomen (ca. neun Jahre vor klinischer Diagnosestellung der Erkrankung). Es zeigte sich unerwarteterweise eine starke Häufung von morphologischen - im Vergleich zu semantischen - Auffälligkeiten und eine extreme hohe, wie auch falsche Verwendung von Anführungsstrichen. Die Ergebnisse erweitern also die bisher nur spärlich vorliegenden Erkenntnisse zum sprachlichen Abbauprozess in der schriftlichen Textproduktion im präklinischem Stadium. Die fluency-Aufgaben für Eigennamen zeigten sich hinsichtlich einem späteren Einsatz als Screeningtool für Demenz als vielversprechend. In der Studie konnte folgendes gezeigt werden: 1) Die fluency für Eigennamen unterscheidet sich tatsächlich von der fluency für Appellativa. 2) Die Leistung der Versuchspersonen variiert in Abhängigkeit vom Eigennamen-fluency-task. Es ist also anzunehmen, dass die Aufgaben tatsächlich eigenständige Aufgaben darstellen und daher unterschiedlich verarbeitet werden. 3) Die Leistungsprofile in den Eigennamen-fluency-tasks von Gesunden und an Alzheimer Demenz Erkrankten unterschieden sich deutlich

What dementia can do to language processing

The thesis at hand focuses on two main aspects: Firstly, the written text production in the preclinical phase of the semantic variant of primary progressive aphasia embedded in a longitudinal study; secondly, on the examination of Proper Names in patients suffering from Alzheimer´s Disease in a fluency task setting. Both studies focus on aspects which have not been subject to scientific research so far. The broad analysis of linguistics in written text production covered the levels of semantics, syntax and morphology and also included the examination of vocabulary and punctuation. The results showed an early onset of symptoms (approximately nine years before the clinical diagnose took place). Most surprising was the finding of an overwhelming amount of morphologic errors and an extreme usage of quotation marks. The results thus deepen the insight in the process of how the syndrome manifests itself in text production even before clinical diagnosis takes place. The fluency tasks for Proper Names proved to be promising for future usage as a screening tool for dementia. It could be shown that Proper Name fluency must be distinguished from Common Name fluency and that performance is different in patients suffering from AD and healthy elderly. Also, performance differs depending on the Proper Name fluency task.Die vorliegende Arbeit konzentriert sich auf zwei Schwerpunkte: Einerseits die schriftliche Textproduktion in der präklinischen Phase bei der semantischen Variante der Primär Progredienten Aphasie im Langzeitverlauf und andererseits die Untersuchung von Eigennamen bei Patienten mit Alzheimer Demenz in Form von fluency tasks. Beide Studien beleuchten Aspekte, zu denen bisher noch kaum wissenschaftliche Erkenntnisse vorliegen. In der umfassenden Studie zur schriftlichen Textproduktion wurden Aspekte aus den Bereichen Semantik, Syntax und Morphologie, sowie Vokabular und Zeichensetzung untersucht. Die Ergbnisse zeigen vor allem einen frühen Onset von ersten Symptomen (ca. neun Jahre vor klinischer Diagnosestellung der Erkrankung). Es zeigte sich unerwarteterweise eine starke Häufung von morphologischen - im Vergleich zu semantischen - Auffälligkeiten und eine extreme hohe, wie auch falsche Verwendung von Anführungsstrichen. Die Ergebnisse erweitern also die bisher nur spärlich vorliegenden Erkenntnisse zum sprachlichen Abbauprozess in der schriftlichen Textproduktion im präklinischem Stadium. Die fluency-Aufgaben für Eigennamen zeigten sich hinsichtlich einem späteren Einsatz als Screeningtool für Demenz als vielversprechend. In der Studie konnte folgendes gezeigt werden: 1) Die fluency für Eigennamen unterscheidet sich tatsächlich von der fluency für Appellativa. 2) Die Leistung der Versuchspersonen variiert in Abhängigkeit vom Eigennamen-fluency-task. Es ist also anzunehmen, dass die Aufgaben tatsächlich eigenständige Aufgaben darstellen und daher unterschiedlich verarbeitet werden. 3) Die Leistungsprofile in den Eigennamen-fluency-tasks von Gesunden und an Alzheimer Demenz Erkrankten unterschieden sich deutlich

A longitudinal linguistic analysis of written text production in a case of semantic variant primary progressive aphasia

The semantic variant of primary progressive aphasia (svPPA) presents with a degradationof semantic knowledge due to atrophy of the anterior temporal lobe and is characterizedby impaired confrontation naming and impaired single-word comprehension. So far, littleis known about the development of symptoms and their order of occurrence in the pre-clinical phase, and information regarding written text production is scarce.We had the unique opportunity to analyze the diary of a man written over a time span of12 years before he was diagnosed with svPPA. We sought to identify the earliest indicatorsof cognitive change in his diary entries, and to track the important changes over time.Based on transcripts of the entries (one week every six months) we assessed the overallstructure, vocabulary, surface dysgraphia and semantic paraphasia, syntax, andmorphology. We found changes in all domains up to seven years before the clinicaldiagnosis. The earliest changes concerned the vocabulary, with decreased variety andincreased use of high frequency words. This was followed by syntactic and morphologicalerrors. We found no increase of surface dysgraphia. Semantic paraphasias increased onlyduring the last three years but characterized the entries of the last year.We were therefore able to further corroborate recentfindings regarding difficulties in themorpho-syntactic domain in this patient group. In this natural context for written textproduction, such errors seem, in addition to changes in vocabulary, to be thefirst errortypes to appear, possibly as a result of compensating for the degradation of semanticrepresentations

Identifying key elements for paediatric advance care planning with parents, healthcare providers and stakeholders: A qualitative study

Background: Although international guidelines recommend discussions about goals of care and treatment options for children with severe and life-limiting conditions, there are still few structured models of paediatric advance care planning. Aim: The study aimed at identifying key components of paediatric advance care planning through direct discussions with all involved parties. Design: The study had a qualitative design with a participatory approach. Participants constituted an advisory board and took part in two transdisciplinary workshops. Data were collected in discussion and dialogue groups and analysed using content analysis. Setting/participants: We included bereaved parents, health care providers and stakeholders of care networks. Results: Key elements were discussions, documentation, implementation, timing and participation of children and adolescents. Parents engage in discussions with facilitators and persons of trust to reach a decision. Documentation constitutes the focus of professionals, who endorse brief recommendations for procedures in case of emergencies, supplemented by larger advance directives. Implementation hindrances include emotional barriers of stakeholders, disagreements between parents and professionals and difficulties with emergency services. Discussion: timing should take into account parental readiness. The intervention should be repeated at regular intervals, considering emerging needs and increasing awareness of families over time. Involving children and adolescents in advance care planning remains a challenge. Conclusion: A paediatric advance care planning intervention should take into account potential pitfalls and barriers including issues related to timing, potential conflicts between parents and professionals, ambiguity towards written advance directives, the role of non-medical carers for paediatric advance care planning implementation, the need to involve the child and the necessity of an iterative process