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.

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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
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article

aFTLD-U presenting with primary progressive aphasia suggestive of non-fluent type with apraxia of speech

Julia Kofler, Óscar López, Ameneh Zare Shahabadi, Riddhi Patira
Neurocase
Genetics and Neurodevelopmental Disorders
article

aFTLD-U presenting with primary progressive aphasia suggestive of non-fluent type with apraxia of speech

Julia Kofler, Óscar López, Ameneh Zare Shahabadi, Riddhi Patira
article en

Abstract

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.

NeurocaseVol. 31(3)
University of Pittsburgh (US)
Quality Education
Openalex Percentile: Top 97%
Genetics and Neurodevelopmental Disorders
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aFTLD-U presenting with primary progressive aphasia suggestive of non-fluent type with apraxia of speech — Julia Kofler, Óscar López, et al. · Neurocase (2025) | TGRS Research Map | TGRS