Identification of an atypical variant of logopenic progressive aphasia

• Published in: Brain and language 2013-11, Vol.127 (2), p.139-144
• Main Authors: Machulda, Mary M., Whitwell, Jennifer L., Duffy, Joseph R., Strand, Edythe A., Dean, Pamela M., Senjem, Matthew L., Jack, Clifford R., Josephs, Keith A.
• Format: Article
• Language: English
• Subjects: Aged; Aphasia, Primary Progressive - complications; Aphasia, Primary Progressive - pathology; Atrophy - pathology; Brain - pathology; Cluster Analysis; Cognition Disorders - complications; Cognition Disorders - pathology; Female; Humans; Logopenic aphasia; Magnetic Resonance Imaging; Male; Middle Aged; Neurocognitive impairment; Neuropsychological Tests; Primary progressive aphasia; Temporoparietal atrophy; Voxel-based morphometry
• ISSN: 0093-934X; 1090-2155
• DOI: 10.1016/j.bandl.2013.02.007

► We found an association between aphasia and neurocognitive severity in lvPPA. ► We found an association between aphasia severity and temporoparietal atrophy in lvPPA. ► We did not find an association between in aphasia severity and disease duration in lvPPA. ► We describe an atypical variant of lvPPA with mild clinical features despite long disease duration. The purpose of this study was to examine the association between aphasia severity and neurocognitive function, disease duration and temporoparietal atrophy in 21 individuals with the logopenic variant of primary progressive aphasia (lvPPA). We found significant correlations between aphasia severity and degree of neurocognitive impairment as well as temporoparietal atrophy; but not disease duration. Cluster analysis identified three variants of lvPPA: (1) subjects with mild aphasia and short disease duration (mild typical lvPPA); (2) subjects with mild aphasia and long disease duration (mild atypical lvPPA); and, (3) subjects with severe aphasia and relatively long disease duration (severe typical lvPPA). All three variants showed temporoparietal atrophy, with the mild atypical group showing the least atrophy despite the longest disease duration. The mild atypical group also showed mild neuropsychological impairment. The subjects with mild aphasia and neuropsychological impairment despite long disease duration may represent a slowly progressive variant of lvPPA.