TY - JOUR
T1 - Predicting clinical decline in progressive agrammatic aphasia and apraxia of speech
AU - Whitwell, Jennifer L.
AU - Weigand, Stephen D.
AU - Duffy, Joseph R.
AU - Clark, Heather M.
AU - Strand, Edythe A.
AU - Machulda, Mary M.
AU - Spychalla, Anthony J.
AU - Senjem, Matthew L.
AU - Jack, Clifford R.
AU - Josephs, Keith A.
N1 - Funding Information:
Supported by the NIH (R01-DC12519 and R01-DC010367).
Funding Information:
J. Whitwell receives funding from NIH grants R01-DC12519, R01-NS089757, R01-AG050603, R01-AG037491, and R21-NS094684. S. Weigand reports no disclosures relevant to the manuscript. J. Duffy receives funding from NIH grants R01-DC12519 and R21-NS094684. H. Clark receives funding from NIH grants R01-DC12519 and R01-NS089757. E. Strand reports no disclosures relevant to the manuscript. M. Machulda receives funding from NIH grants R01-DC12519 and R01-AG050603. A. Spychalla and M. Senjem report no disclosures relevant to the manuscript. C. Jack serves as a consultant for Eli Lily and receives research funding from the NIH (R01-AG011378, RO1-AG041851, RO1-AG037551, U01-HL096917, U01-AG032438, U01-AG024904) and the Alexander Family Alzheimer’s Disease Research Professorship of the Mayo Foundation. K. Josephs receives funding from NIH grants R01-AG037491, R01-NS089757, R01-DC12519, R01-AG050603, and R21-NS094684. Go to Neurology.org for full disclosures.
Publisher Copyright:
© 2017 American Academy of Neurology.
PY - 2017
Y1 - 2017
N2 - Objective: To determine whether baseline clinical and MRI features predict rate of clinical decline in patients with progressive apraxia of speech (AOS). Methods: Thirty-four patients with progressive AOS, with AOS either in isolation or in the presence of agrammatic aphasia, were followed up longitudinally for up to 4 visits, with clinical testing and MRI at each visit. Linear mixed-effects regression models including all visits (n = 94) were used to assess baseline clinical and MRI variables that predict rate of worsening of aphasia, motor speech, parkinsonism, and behavior. Clinical predictors included baseline severity and AOS type. MRI predictors included baseline frontal, premotor, motor, and striatal gray matter volumes. Results: More severe parkinsonism at baseline was associated with faster rate of decline in parkinsonism. Patients with predominant sound distortions (AOS type 1) showed faster rates of decline in aphasia and motor speech, while patients with segmented speech (AOS type 2) showed faster rates of decline in parkinsonism. On MRI, we observed trends for fastest rates of decline in aphasia in patients with relatively small left, but preserved right, Broca area and precentral cortex. Bilateral reductions in lateral premotor cortex were associated with faster rates of decline of behavior. No associations were observed between volumes and decline in motor speech or parkinsonism. Conclusions: Rate of decline of each of the 4 clinical features assessed was associated with different baseline clinical and regional MRI predictors. Our findings could help improve prognostic estimates for these patients.
AB - Objective: To determine whether baseline clinical and MRI features predict rate of clinical decline in patients with progressive apraxia of speech (AOS). Methods: Thirty-four patients with progressive AOS, with AOS either in isolation or in the presence of agrammatic aphasia, were followed up longitudinally for up to 4 visits, with clinical testing and MRI at each visit. Linear mixed-effects regression models including all visits (n = 94) were used to assess baseline clinical and MRI variables that predict rate of worsening of aphasia, motor speech, parkinsonism, and behavior. Clinical predictors included baseline severity and AOS type. MRI predictors included baseline frontal, premotor, motor, and striatal gray matter volumes. Results: More severe parkinsonism at baseline was associated with faster rate of decline in parkinsonism. Patients with predominant sound distortions (AOS type 1) showed faster rates of decline in aphasia and motor speech, while patients with segmented speech (AOS type 2) showed faster rates of decline in parkinsonism. On MRI, we observed trends for fastest rates of decline in aphasia in patients with relatively small left, but preserved right, Broca area and precentral cortex. Bilateral reductions in lateral premotor cortex were associated with faster rates of decline of behavior. No associations were observed between volumes and decline in motor speech or parkinsonism. Conclusions: Rate of decline of each of the 4 clinical features assessed was associated with different baseline clinical and regional MRI predictors. Our findings could help improve prognostic estimates for these patients.
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U2 - 10.1212/WNL.0000000000004685
DO - 10.1212/WNL.0000000000004685
M3 - Article
C2 - 29093069
AN - SCOPUS:85038016008
VL - 89
SP - 2271
EP - 2279
JO - Neurology
JF - Neurology
SN - 0028-3878
IS - 22
ER -