A molecular pathology, neurobiology, biochemical, genetic and neuroimaging study of progressive apraxia of speech.

Nature Communications
Keith A JosephsJennifer L Whitwell

Abstract

Progressive apraxia of speech is a neurodegenerative syndrome affecting spoken communication. Molecular pathology, biochemistry, genetics, and longitudinal imaging were investigated in 32 autopsy-confirmed patients with progressive apraxia of speech who were followed over 10 years. Corticobasal degeneration and progressive supranuclear palsy (4R-tauopathies) were the most common underlying pathologies. Perceptually distinct speech characteristics, combined with age-at-onset, predicted specific 4R-tauopathy; phonetic subtype and younger age predicted corticobasal degeneration, and prosodic subtype and older age predicted progressive supranuclear palsy. Phonetic and prosodic subtypes showed differing relationships within the cortico-striato-pallido-nigro-luysial network. Biochemical analysis revealed no distinct differences in aggregated 4R-tau while tau H1 haplotype frequency (69%) was lower compared to 1000+ autopsy-confirmed 4R-tauopathies. Corticobasal degeneration patients had faster rates of decline, greater cortical degeneration, and shorter illness duration than progressive supranuclear palsy. These findings help define the pathobiology of progressive apraxia of speech and may have consequences for development of 4R-tau t...Continue Reading

References

Jul 1, 1992·Journal of Neurology, Neurosurgery, and Psychiatry·E BroussolleG Chazot
Jan 1, 1991·Acta Neuropathologica·H Braak, E Braak
Aug 1, 1994·Journal of Neuroscience Methods·R CrookK Duff
Jun 13, 2001·Neurology·D W Dickson
Nov 1, 2001·Applied Neuropsychology·A S LoonstraA H Sellers
Nov 15, 2002·Journal of Neuropathology and Experimental Neurology·D W DicksonUNKNOWN Office of Rare Diseases of the National Institutes of Health
Dec 1, 1962·Brain : a Journal of Neurology·E DE RENZI, L A VIGNOLO
Apr 20, 2004·Brain : a Journal of Neurology·Argye E HillisKristin Maurer
Feb 16, 2006·Neurology·Stewart A FactorJiang Qian
Apr 15, 2006·Brain : a Journal of Neurology·Keith A JosephsRonald C Petersen
May 13, 2006·Journal of Neuropathology and Experimental Neurology·Keith A JosephsDennis W Dickson
Nov 14, 2006·Neurobiology of Aging·Keith A JosephsClifford R Jack
Aug 2, 2007·American Journal of Speech-language Pathology·Joseph R DuffyEdythe A Strand
Dec 20, 2007·Alzheimer Disease and Associated Disorders·Jennifer M OgarMaria Luisa Gorno-Tempini
Aug 25, 2009·American Journal of Alzheimer's Disease and Other Dementias·Sandra WeintraubCynthia K Thompson
Oct 13, 2009·NeuroImage·Brian B AvantsJames C Gee
Jun 7, 2011·Acta Neuropathologica·Ian R A MackenzieVirginia M Y Lee
Jul 2, 2011·Journal of Molecular Neuroscience : MN·Dennis W DicksonKeith A Josephs
Aug 4, 2011·Brain : a Journal of Neurology·Katya RascovskyBruce L Miller
Mar 3, 2012·Brain : a Journal of Neurology·Keith A JosephsJennifer L Whitwell
Jan 30, 2013·Neurology·Melissa J ArmstrongWilliam J Weiner
Jun 12, 2013·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·J L WhitwellK A Josephs
Sep 3, 2013·Acta Neuropathologica·Zeshan AhmedGabor G Kovacs
Dec 20, 2013·Neurology·Francesca CasoMaria Luisa Gorno-Tempini
Jan 5, 2014·Acta Neuropathologica·Marka van BlitterswijkRosa Rademakers
Feb 22, 2014·Brain and Language·Jonathan Graff-RadfordKeith A Josephs
Apr 15, 2014·Neurology·Hugo BothaKeith A Josephs
Aug 6, 2014·Journal of Communication Disorders·Edythe A StrandKeith Josephs
Aug 13, 2014·Brain : a Journal of Neurology·Keith A JosephsJennifer L Whitwell
Apr 25, 2015·Stroke; a Journal of Cerebral Circulation·Alexandra BasilakosJulius Fridriksson
Jun 17, 2015·Journal of Neurology·Jennifer L WhitwellKeith A Josephs
Jun 25, 2015·NeuroImage. Clinical·Anneliese B NewKirrie J Ballard
Oct 25, 2016·Cortex; a Journal Devoted to the Study of the Nervous System and Behavior·Jennifer L WhitwellKeith A Josephs

❮ Previous
Next ❯

Citations

Oct 12, 2021·Movement Disorders : Official Journal of the Movement Disorder Society·Fatma Ozlem HokelekliKeith A Josephs

❮ Previous
Next ❯

Software Mentioned

R
FMRIB58
Access
CortexID
Eve
Advanced Normalization Tools ( ANTs )
QuantStudio Real - Time PCR
BrainNet Viewer
REDCap
ANTs

Related Concepts

Related Feeds

Adrenocortical Carcinoma

Adrenocortical carcinoma (ACC) is a rare malignancy of the adrenal cortex, associated with a generally dismal prognosis owing to its aggressive behavior. Here are the latest discoveries pertaining to this disease.

Related Papers

European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies
Jennifer L WhitwellKeith A Josephs
Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine
Mitsunori Morimatsu
© 2021 Meta ULC. All rights reserved