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Rates of Amyloid Imaging Positivity in Patients With Primary Progressive Aphasia.
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In: JAMA neurology, vol 75, iss 3 (2018)
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Rates of Amyloid Imaging Positivity in Patients With Primary Progressive Aphasia.
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Santos-Santos, Miguel A; Rabinovici, Gil D; Iaccarino, Leonardo; Ayakta, Nagehan; Tammewar, Gautam; Lobach, Iryna; Henry, Maya L; Hubbard, Isabel; Mandelli, Maria Luisa; Spinelli, Edoardo; Miller, Zachary A; Pressman, Peter S; O'Neil, James P; Ghosh, Pia; Lazaris, Andreas; Meyer, Marita; Watson, Christa; Yoon, Soo Jin; Rosen, Howard J; Grinberg, Lea; Seeley, William W; Miller, Bruce L; Jagust, William J; Gorno-Tempini, Maria Luisa
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In: JAMA neurology, vol 75, iss 3 (2018)
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Abstract:
Importance:The ability to predict the pathology underlying different neurodegenerative syndromes is of critical importance owing to the advent of molecule-specific therapies. Objective:To determine the rates of positron emission tomography (PET) amyloid positivity in the main clinical variants of primary progressive aphasia (PPA). Design, Setting, and Participants:This prospective clinical-pathologic case series was conducted at a tertiary research clinic specialized in cognitive disorders. Patients were evaluated as part of a prospective, longitudinal research study between January 2002 and December 2015. Inclusion criteria included clinical diagnosis of PPA; availability of complete speech, language, and cognitive testing; magnetic resonance imaging performed within 6 months of the cognitive evaluation; and PET carbon 11-labeled Pittsburgh Compound-B or florbetapir F 18 brain scan results. Of 109 patients referred for evaluation of language symptoms who underwent amyloid brain imaging, 3 were excluded because of incomplete language evaluations, 5 for absence of significant aphasia, and 12 for presenting with significant initial symptoms outside of the language domain, leaving a cohort of 89 patients with PPA. Main Outcomes and Measures:Clinical, cognitive, neuroimaging, and pathology results. Results:Twenty-eight cases were classified as imaging-supported semantic variant PPA (11 women [39.3%]; mean [SD] age, 64 [7] years), 31 nonfluent/agrammatic variant PPA (22 women [71.0%]; mean [SD] age, 68 [7] years), 26 logopenic variant PPA (17 women [65.4%]; mean [SD] age, 63 [8] years), and 4 mixed PPA cases. Twenty-four of 28 patients with semantic variant PPA (86%) and 28 of 31 patients with nonfluent/agrammatic variant PPA (90%) had negative amyloid PET scan results, while 25 of 26 patients with logopenic variant PPA (96%) and 3 of 4 mixed PPA cases (75%) had positive scan results. The amyloid positive semantic variant PPA and nonfluent/agrammatic variant PPA cases with available autopsy data (2 of 4 and 2 of 3, respectively) all had a primary frontotemporal lobar degeneration and secondary Alzheimer disease pathologic diagnoses, whereas autopsy of 2 patients with amyloid PET-positive logopenic variant PPA confirmed Alzheimer disease. One mixed PPA patient with a negative amyloid PET scan had Pick disease at autopsy. Conclusions and Relevance:Primary progressive aphasia variant diagnosis according to the current classification scheme is associated with Alzheimer disease biomarker status, with the logopenic variant being associated with carbon 11-labeled Pittsburgh Compound-B positivity in more than 95% of cases. Furthermore, in the presence of a clinical syndrome highly predictive of frontotemporal lobar degeneration pathology, biomarker positivity for Alzheimer disease may be associated more with mixed pathology rather than primary Alzheimer disease.
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Keyword:
2.1 Biological and endogenous factors; 4.1 Discovery and preclinical testing of markers and technologies; 4.2 Evaluation of markers and technologies; Acquired Cognitive Impairment; Aged; Aging; Alzheimer's Disease; Alzheimer's Disease including Alzheimer's Disease Related Dementias (AD/ADRD); Alzheimer's Disease Related Dementias (ADRD); Amyloid; Aniline Compounds; Aphasia; Biomedical Imaging; Brain; Brain Disorders; Clinical Research; Clinical Trials and Supportive Activities; Dementia; Ethylene Glycols; Female; Frontotemporal Dementia (FTD); Humans; Imaging; Longitudinal Studies; Magnetic Resonance Imaging; Male; Middle Aged; Neurodegenerative; Neurological; Neuropsychological Tests; Neurosciences; Positron-Emission Tomography; Primary Progressive; Rare Diseases; Retrospective Studies; Severity of Illness Index; Thiazoles; Three-Dimensional
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URL: https://escholarship.org/uc/item/1hm128x5
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