aFTLD-U presenting with primary progressive aphasia suggestive of non-fluent type with apraxia of speech
Atypical frontotemporal lobar degeneration with ubiquitin-positive inclusions (aFTLD-U) is a rare subtype of Frontotemporal Lobar Degeneration. aFTLD-U is reported with a typically young onset behavioral variant of frontotemporal dementia syndrome, more specifically with younger age, predominant hyperorality, obsessive-compulsive features, and severe caudate atrophy. Very few cases have been reported with prominent language impairment with no major behavioral features. We present another such rare case of aFTLD-U presenting with prominent language impairment adding to the phenotypic spectrum, with language deficits observed primarily in spoken language seen in apraxia of speech associated with non-fluent primary progressive aphasia.
Authors
- Julia Kofler (ORCID: https://orcid.org/0000-0003-4298-7328)
- Óscar López (ORCID: https://orcid.org/0000-0003-2896-6993)
- Ameneh Zare Shahabadi
- Riddhi Patira
Institutions
- University of Pittsburgh (US)
Publication Details
- Journal
- Neurocase
- Published
- 2025-02-11
- DOI
- https://doi.org/10.1080/13554794.2025.2464549
- Primary Topic
- Genetics and Neurodevelopmental Disorders
- Type
- article
- Field-Weighted Citation Impact
- 0.00