Objective: This review describes the relatively small body of neuropsychological and cognitive research conducted over the past 100 years focused on theoretical models explaining the neurocognitive processes that support everyday functioning and the breakdown of functional abilities in the face of neurological damage or disease. Method: The historical roots of the theories of everyday activities based on direct observation of behavior in neurology and diary reports of everyday errors in cognitive psychology are presented, followed by a review of the empirical findings and resulting theoretical conceptualizations from case studies and group studies of various clinical populations in neuropsychology. Results: We conclude with a new framework (the goal-control model) that integrates the most recent empirical findings in neuropsychology with mechanisms proposed by cognitive models. Conclusions: The goal-control model offers empirically supported solutions to understanding and predicting functioning in the real world. This new model generates testable predictions for future research and provides guidance for clinical assessment and interventions.

Background: While primary progressive aphasia (PPA) is associated with frontotemporal lobar degeneration (FTLD) pathology due to tau or TDP, clinical-pathological studies also demonstrate many cases have Alzheimer's disease (AD) pathology. The logopenic variant of PPA (lvPPA) is most often associated with AD pathology, but this has proven to be the least reliable PPA to diagnose using published clinical criteria. In this study, we used cerebrospinal fluid (CSF) analytes to identify patients with likely AD pathology, and relate phenotypic features of lvPPA to CSF. Methods: We studied 46 PPA patients who had available CSF analytes, including 26 with a clinical diagnosis of lvPPA, 9 with non-fluent/agrammatic variant (naPPA), and 11 with semantic variant (svPPA). We identified patients with likely AD pathology using amyloid-beta 1–42 (Aβ 1−42 ) < 192 pg/ml and assessed MRI gray matter atrophy in these patients. Results: We found that 23 (49%) of 46 PPA patients have a low CSF Aβ 1−42 level consistent with AD pathology. Twenty-one (91%) of 23 patients had a lvPPA phenotype, and 18 (79%) of 23 cases with an elevated CSF Aβ 1−42 level did not have a lvPPA phenotype. Patients with a lvPPA phenotype demonstrated GM atrophy in the left lateral temporal lobe, and this was also seen in those with a CSF Aβ 1−42 level < 192 pg/ml. Conclusion: The lvPPA clinical phenotype may be a useful screen for CSF analytes that are a surrogate for likely AD pathology, and may help establish eligibility of these patients for disease-modifying treatment trials.

Purpose Primary progressive aphasia (PPA) and the amnestic variant of Alzheimer's disease (AD) are neurodegenerative conditions characterized by a profound loss of functional communication abilities. Communicative impairment in AD and PPA is especially apparent in the domain of naming common objects and familiar faces. We evaluated the effectiveness of a language intervention targeting maintenance of an individualized core vocabulary in a longitudinal cohort of older adults experiencing either PPA or AD. Method PPA ( n = 9) and AD ( n = 1) patients were administered a semantically based language treatment for up to 2 years. Patients repeatedly named and generated semantic features for a personalized lexicon consisting of 100 words. We evaluated naming accuracy and off-line neuropsychological measures at four successive timepoints. Naming accuracy was assessed in patients ( n = 7) who completed at least three recurrent evaluations. Off-line neuropsychological performance was assessed across timepoints in all patients. Results Patients demonstrated relative preservation of naming trained words relative to a steep decline for untrained (control) words. The greatest decrements were observed for naming people relative to objects. Conclusion These results suggest that consistent training of a finite set of words can protect a core lexicon composed of crucial target concepts (e.g., a spouse's name). We discuss potential benefits and clinical implications of maintenance-based approaches to promoting language functioning in the context of neurodegeneration.

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