Affiliations: [a] Department of Neurology, Center for Brain/Mind Medicine, Brigham and Women's Hospital, Boston, MA, USA | [b] Department of Psychiatry, Brigham and Women's Hospital, Boston, MA, USA | [c] Department of Psychiatry, Beth Israel Deaconess Medical Center, Boston, MA, USA | [d] Frontotemporal Dementia Unit, Departments of Neurology and Psychiatry, Massachusetts General Hospital, Boston, MA, USA | [e] Athinoula A. Martinos Center for Biomedical Imaging, Charlestown, MA, USA
Correspondence:
[*]
Correspondence to: Kirk R. Daffner, M.D., Division of Cognitive and Behavioral Neurology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA 02115, USA. Tel.: +1 617 732 8060; Fax: +1 617 738 9122; E-mail: kdaffner@partners.org.
Note: [1] These authors contributed equally to this manuscript.
Abstract: Primary progressive aphasia (PPA) is a language predominant neurodegenerative disorder that has three recognized variants: nonfluent/agrammatic, semantic, and logopenic. This report describes a 60-year-old man who presented with a progressive decline in verbal output that does not fit the currently accepted PPA subtypes. The patient exhibited a paucity of verbal output and impaired phonemic fluency with minimal associated language, cognitive, or behavioral deficits. Focal cortical thinning/hypometabolism of the left superior frontal region and a cerebrospinal fluid profile not consistent with Alzheimer's disease pathology were identified. This case of isolated progressive dynamic aphasia extends the current boundaries of PPA diagnostic variants.
Keywords: Cerebrospinal fluid, magnetic resonance imaging, neurodegenerative disease, PET scan, primary progressive aphasia
