Riddhi Patira, Ameneh Zare Shahabadi, Oscar Lopez, Julia Kofler
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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.
期刊介绍:
Neurocase is a rapid response journal of case studies and innovative group studies in neuropsychology, neuropsychiatry and behavioral neurology that speak to the neural basis of cognition. Four types of manuscript are considered for publication: single case investigations that bear directly on issues of relevance to theoretical issues or brain-behavior relationships; group studies of subjects with brain dysfunction that address issues relevant to the understanding of human cognition; reviews of important topics in the domains of neuropsychology, neuropsychiatry and behavioral neurology; and brief reports (up to 2500 words) that replicate previous reports dealing with issues of considerable significance. Of particular interest are investigations that include precise anatomical localization of lesions or neural activity via imaging or other techniques, as well as studies of patients with neurodegenerative diseases, since these diseases are becoming more common as our population ages. Topic reviews are included in most issues.