Objective: To study and compare the neuropsychological profile of patients with corticobasal degeneration (CBD), progressive supranuclear palsy (PSP), non-fluent progressive aphasia (NFPA) and frontotemporal behavioral dementia (b-FTD).

Background: Frontotemporal lobar degeneration encompasses three different syndromes, with clinical and pathologic commonalities, making diagnosis difficult in early stages. Three subtypes are recognized: frontotemporal dementia and its three variants, corticobasal syndrome and supranuclear palsy syndrome. Although there is an overlap amongst the different subtypes of frontotemporal lobar degeneration, neuropsychological profiles can help identify subtypes and discriminate frontotemporal lobar degeneration from other forms of dementia.

Methods: A neuropsychological assessment was performed in a total of 14 CBD patients, 21 with PSP, 15 with NFPA and 33 with b-FTD. Comparisons of the scores on visual and episodic memory tests (ADAS, Buschke and memory of figures), executive function (Stroop, TrailMaking, Raven and memory of figures), language (Boston) and visuospatial function (copy of figures) were analyzed. Statistical analysis was performed using non-parametric tests (Kruskal.Wallis)

Results: NFPA patients scored better in all domains, although they performed poorly on MMSE than either CBD or PSP. Patients with b-FTD performed worse on MMSE, language and memory than patients with CBD and PSP, but scored better on the scale for activities of daily living. Patients with PSP obtained the lowest score in phonetic verbal fluency and lowest score in Stroop versus b-FTD. Patients with CBD and PSP performed significantly better in cued recall than patients with NFPA and b-FTD.

Conclusions: NFPA patients perform globally better than the rest of the groups. Patients with b-FTD perform worse in memory and language. CBD and PSP significantly improved in memory tests, with cueing. 

References: Kertesz A, Martinez-Lage P, Davidson W, Munoz DG. The corticobasal degeneration syndrome overlaps progressive aphasia and frontotemporal dementia. Neurology 2000; 55: 1368-1375.

Lagarde J, Valabregue R, Corvol JC, Pineau F, Le Ber I, Vidailhet M et al. Are frontal cognitive and atrophy patterns different in PSP and bvFTD? A comparative neuropsychological and VBM Study. PLoS One 2013; 8: e80353.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/neuropsychological-profile-of-corticobasal-degeneration-progressive-supranuclear-palsy-and-frontotemporal-dementia-patients/