title: Primary progressive aphasia accompanied by environmental sound agnosia: a neuropsychological, MRI and PET study.
abstract: As part of the frontotemporal dementias, primary progressive aphasia (PPA) is typically characterized by nonfluent speech with paraphasias, but there is growing evidence that also a fluent variant of PPA exists. We describe a patient suffering from PPA who adds to the broad clinical spectrum of this disorder. Moreover, we report for the first time that PPA may be associated with severe impairment in meaningful nonverbal sound recognition (environmental sound agnosia). These neuropsychological findings were found to be associated with distinct focal alterations in functional and structural neuroimaging.
authors.affiliation: Department of Neurology, University of Ulm, Oberer Eselsberg 45, 89081 Ulm, Germany. ingo.uttner@uni-ulm.de
authors.forename: Ingo
authors.initials: I
authors.lastname: Uttner
authors.forename: Felix M
authors.initials: FM
authors.lastname: Mottaghy
authors.forename: Herbert
authors.initials: H
authors.lastname: Schreiber
authors.forename: Axel
authors.initials: A
authors.lastname: Riecker
authors.forename: Albert C
authors.initials: AC
authors.lastname: Ludolph
authors.forename: Jan
authors.initials: J
authors.lastname: Kassubek
mesh_terms: D000368:Aged; D001037:Aphasia / Q000175:diagnosis* / Q000209:etiology*; D001308:Auditory Perceptual Disorders / Q000175:diagnosis / Q000209:etiology*; D001921:Brain / Q000378:metabolism* / Q000473:pathology*; D003704:Dementia / Q000150:complications* / Q000175:diagnosis*; D018450:Disease Progression; D004777:Environment; D005260:Female; D019788:Fluorodeoxyglucose F18 / Q000493:pharmacokinetics; D006801:Humans; D008279:Magnetic Resonance Imaging; D009483:Neuropsychological Tests; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals / Q000493:pharmacokinetics; D021641:Recognition, Psychology; D012720:Severity of Illness Index
chemical_list: D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18