Jason D Warren1, Chris Jd Hardy2, Justina Ruksenaite1, Anna Volkmer3, Jessica Jiang1, Jeremy Cs Johnson1, Charles R Marshall4. 1. Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, 8 - 11 Queen Square, London, WC1N 3BG, UK. 2. Dementia Research Centre, Department of Neurodegenerative Disease, UCL Queen Square Institute of Neurology, University College London, 8 - 11 Queen Square, London, WC1N 3BG, UK. chris.hardy@ucl.ac.uk. 3. Division of Psychology and Language Sciences, University College London, London, UK. 4. Preventive Neurology Unit, Wolfson Institute of Preventive Medicine, Queen Mary University of London, London, UK.
Abstract
PURPOSE OF REVIEW: The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers. RECENT FINDINGS: Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering 'fragmentary' syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed. New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.
PURPOSE OF REVIEW: The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers. RECENT FINDINGS: Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering 'fragmentary' syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed. New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.
Authors: Keith A Josephs; Joseph R Duffy; Edythe A Strand; Mary M Machulda; Matthew L Senjem; Val J Lowe; Clifford R Jack; Jennifer L Whitwell Journal: Neurology Date: 2013-06-26 Impact factor: 9.910
Authors: Tarun D Singh; Joseph R Duffy; Edythe A Strand; Mary M Machulda; Jennifer L Whitwell; Keith A Josephs Journal: Dement Geriatr Cogn Disord Date: 2015-01-21 Impact factor: 2.959
Authors: Jonathan D Rohrer; Tammaryn Lashley; Jonathan M Schott; Jane E Warren; Simon Mead; Adrian M Isaacs; Jonathan Beck; John Hardy; Rohan de Silva; Elizabeth Warrington; Claire Troakes; Safa Al-Sarraj; Andrew King; Barbara Borroni; Matthew J Clarkson; Sebastien Ourselin; Janice L Holton; Nick C Fox; Tamas Revesz; Martin N Rossor; Jason D Warren Journal: Brain Date: 2011-09 Impact factor: 13.501
Authors: Keith A Josephs; Joseph R Duffy; Edythe A Strand; Mary M Machulda; Matthew L Senjem; Ankit V Master; Val J Lowe; Clifford R Jack; Jennifer L Whitwell Journal: Brain Date: 2012-03-01 Impact factor: 13.501
Authors: Jonathan D Rohrer; Ione O C Woollacott; Katrina M Dick; Emilie Brotherhood; Elizabeth Gordon; Alexander Fellows; Jamie Toombs; Ronald Druyeh; M Jorge Cardoso; Sebastien Ourselin; Jennifer M Nicholas; Niklas Norgren; Simon Mead; Ulf Andreasson; Kaj Blennow; Jonathan M Schott; Nick C Fox; Jason D Warren; Henrik Zetterberg Journal: Neurology Date: 2016-08-31 Impact factor: 9.910
Authors: Charles R Marshall; Chris J D Hardy; Anna Volkmer; Lucy L Russell; Rebecca L Bond; Phillip D Fletcher; Camilla N Clark; Catherine J Mummery; Jonathan M Schott; Martin N Rossor; Nick C Fox; Sebastian J Crutch; Jonathan D Rohrer; Jason D Warren Journal: J Neurol Date: 2018-02-01 Impact factor: 4.849
Authors: Maria Serpente; Laura Ghezzi; Chiara Fenoglio; Francesca R Buccellato; Giorgio G Fumagalli; Emanuela Rotondo; Marina Arcaro; Andrea Arighi; Daniela Galimberti Journal: Int J Mol Sci Date: 2022-07-30 Impact factor: 6.208
Authors: Siddharth Ramanan; Muireann Irish; Karalyn Patterson; James B Rowe; Maria Luisa Gorno-Tempini; Matthew A Lambon Ralph Journal: Brain Date: 2022-09-14 Impact factor: 15.255
Authors: Jessica Jiang; Elia Benhamou; Sheena Waters; Jeremy C S Johnson; Anna Volkmer; Rimona S Weil; Charles R Marshall; Jason D Warren; Chris J D Hardy Journal: Brain Sci Date: 2021-03-20