Limb and face apraxias in frontotemporal dementia: A systematic scoping review

To investigate the literature for frequencies, profiles and neural correlates of limb and face apraxias in frontotemporal dementia (FTD).

The search conducted in Ovid Medline, PsycINFO and Scopus yielded 487 non-duplicate records, and 43 were included in the final analysis.

Apraxias are evident in diverse forms in all clinical variants of FTD within the first four years of the disease. Face apraxia and productive limb apraxia co-occur in the behavioural and nonfluent variants. The logopenic variant resembles Alzheimer's disease in terms of pronounced parietal limb apraxia and absence of face apraxia. The semantic variant exhibits conceptual praxis deficits together with relatively preserved imitation skills. Concerning the genetic variants of FTD, productive limb apraxia is common among carriers of the progranulin gene mutation, and subtle gestural alterations have been documented among carriers of the chromosome 9 open reading frame 72 gene mutation before the expected disease onset. The data on neural correlations suggest that the breakdown of praxis results from bilateral cortical and subcortical damage in FTD and that Alzheimer-type pathology of the cerebrospinal fluid increases the severity of limb apraxia in all of the variants. Face apraxia correlates with degeneration of the medial and superior frontal cortices.

Each of the clinical variants of FTD exhibits a characteristic profile of apraxias that may support early differentiation between the variants and from Alzheimer's disease. However, the screening procedures developed for stroke populations seem insufficient, and a multifaceted assessment tool is needed. Although valid and practical tests already exist for dementia populations, a concise selection of test items that covers all of the critical domains is called for.