Agraphia in patients with frontotemporal dementia and parkinsonism linked to chromosome 17 with P301LMAPTmutation: dysexecutive, aphasic, apraxic or spatial phenomenon?

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.

Frontotemporal lobar degeneration due to P301L tau mutation showing apathy and severe frontal atrophy but lacking other behavioral changes: A case report and literature review

“…Of all 31 cases that we reviewed, only one case (3.2%; Case 20 in Table ) presented with apathy as the sole initial symptom: this was a male patient who initially developed apathy without other obvious behavioral changes at the age of 49 years. However, unlike our case, depression characterized by suicidal ideation developed 1 year later . At 55 years, he developed L‐dopa‐responsive parkinsonism (unilateral resting tremor and hypomimia).…”

“…The most frequently noted initial symptom was personality change (13 cases, 41.9%). Behavioral change was the second most common (10 cases, 32.3%, and personality change was also noted in eight of these 10 cases) . The third most common was memory impairment, being observed in six cases (19.4%) .…”

“…Parkinsonism was also often noted as an onset symptom (five cases, 16.1%) . Other initial symptoms described were word‐finding problems (two cases), semantic memory impairment (one case), poor concentration (one case), subtle attention deficits (one case), alien limb sign (one case), irritability (one case), aggression (one case) and persecutory delusion (one case) . Of all 31 cases that we reviewed, only one case (3.2%; Case 20 in Table ) presented with apathy as the sole initial symptom: this was a male patient who initially developed apathy without other obvious behavioral changes at the age of 49 years.…”

Cognitive Performance Deficits and Dysgraphia in Alzheimer’s Disease Patients

“…Furthermore, clear differences between AD patients and healthy control individuals have been found for visuomotor task measures demonstrating large effect size deficits by AD patients especially with visuomotor task progression through its varying conditions [51]. Sitek et al [52] have observed dysgraphia, primarily dysexecutive agraphia, in patients with frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). Patients presenting 'dysexecutive' agraphia show not only difficulties in maintaining the effort inherent to writing but appear to lack the ability to organize their thoughts for expression in the written text.…”

Cognitive Performance Deficits and Dysgraphia in Alzheimer’s Disease Patients

“…Furthermore, clear differences between AD patients and healthy control individuals have been found for visuomotor task measures demonstrating large effect size deficits by AD patients especially with visuomotor task progression through its varying conditions [49]. Sitek et al (2014) [50] have observed dysgraphia, primarily 'dysexecutive. agraphia, in patients with frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17).…”