Marina Buciuc1, Joseph R Duffy1, Mary M Machulda1, Jonathan Graff-Radford1, Nha Trang Thu Pham1, Peter R Martin1, Matthew L Senjem1, Clifford R Jack1, Nilüfer Ertekin-Taner1, Dennis W Dickson1, Val J Lowe1, Jennifer L Whitwell1, Keith Anthony Josephs2. 1. From the Departments of Neurology (M.B., J.R.D., J.G.-R., K.A.J.), Psychiatry and Psychology (M.M.M.), Radiology (N.T.T.P., M.L.S., C.R.J., V.J.L., J.L.W.), Health Science Research (P.R.M.), and Information Technology (M.L.S.), Mayo Clinic, Rochester, MN; and Departments of Neurology (N.E.-T.) and Neuroscience (N.E.-T., D.W.D.), Mayo Clinic, Jacksonville, FL. 2. From the Departments of Neurology (M.B., J.R.D., J.G.-R., K.A.J.), Psychiatry and Psychology (M.M.M.), Radiology (N.T.T.P., M.L.S., C.R.J., V.J.L., J.L.W.), Health Science Research (P.R.M.), and Information Technology (M.L.S.), Mayo Clinic, Rochester, MN; and Departments of Neurology (N.E.-T.) and Neuroscience (N.E.-T., D.W.D.), Mayo Clinic, Jacksonville, FL. josephs.keith@mayo.edu.
Abstract
OBJECTIVE: To assess and compare demographic, clinical, neuroimaging, and pathologic characteristics of a cohort of patients with right hemisphere-predominant vs left hemisphere-predominant logopenic progressive aphasia (LPA). METHODS: This is a case-control study of patients with LPA who were prospectively followed at Mayo Clinic and underwent [18F]-fluorodeoxyglucose (FDG) PET scan. Patients were classified as rLPA if right temporal lobe metabolism was ≥1 SD lower than left temporal lobe metabolism. Patients with rLPA were frequency-matched 3:1 to typical left-predominant LPA based on degree of asymmetry and severity of temporal lobe metabolism. Patients were compared on clinical, imaging (MRI, FDG-PET, β-amyloid, and tau-PET), and pathologic characteristics. RESULTS: Of 103 prospectively recruited patients with LPA, 8 (4 female) were classified as rLPA (7.8%); all patients with rLPA were right-handed. Patients with rLPA had milder aphasia based on the Western Aphasia Battery-Aphasia Quotient (p = 0.04) and less frequent phonologic errors (p = 0.015). Patients with rLPA had shorter survival compared to typical LPA: hazard ratio 4.0 (1.2-12.9), p = 0.02. There were no other differences in demographics, handedness, genetics, or neurologic or neuropsychological tests. Compared to the 24 frequency-matched patients with typical LPA, patients with rLPA showed greater frontotemporal hypometabolism of the nondominant hemisphere on FDG-PET and less atrophy in amygdala and hippocampus of the dominant hemisphere. Autopsy evaluation revealed a similar distribution of pathologic findings in both groups, with Alzheimer disease pathologic changes being the most frequent pathology. CONCLUSIONS: rLPA is associated with less severe aphasia but has shorter survival from reported symptom onset than typical LPA, possibly related to greater involvement of the nondominant hemisphere.
OBJECTIVE: To assess and compare demographic, clinical, neuroimaging, and pathologic characteristics of a cohort of patients with right hemisphere-predominant vs left hemisphere-predominant logopenic progressive aphasia (LPA). METHODS: This is a case-control study of patients with LPA who were prospectively followed at Mayo Clinic and underwent [18F]-fluorodeoxyglucose (FDG) PET scan. Patients were classified as rLPA if right temporal lobe metabolism was ≥1 SD lower than left temporal lobe metabolism. Patients with rLPA were frequency-matched 3:1 to typical left-predominant LPA based on degree of asymmetry and severity of temporal lobe metabolism. Patients were compared on clinical, imaging (MRI, FDG-PET, β-amyloid, and tau-PET), and pathologic characteristics. RESULTS: Of 103 prospectively recruited patients with LPA, 8 (4 female) were classified as rLPA (7.8%); all patients with rLPA were right-handed. Patients with rLPA had milder aphasia based on the Western Aphasia Battery-Aphasia Quotient (p = 0.04) and less frequent phonologic errors (p = 0.015). Patients with rLPA had shorter survival compared to typical LPA: hazard ratio 4.0 (1.2-12.9), p = 0.02. There were no other differences in demographics, handedness, genetics, or neurologic or neuropsychological tests. Compared to the 24 frequency-matched patients with typical LPA, patients with rLPA showed greater frontotemporal hypometabolism of the nondominant hemisphere on FDG-PET and less atrophy in amygdala and hippocampus of the dominant hemisphere. Autopsy evaluation revealed a similar distribution of pathologic findings in both groups, with Alzheimer disease pathologic changes being the most frequent pathology. CONCLUSIONS: rLPA is associated with less severe aphasia but has shorter survival from reported symptom onset than typical LPA, possibly related to greater involvement of the nondominant hemisphere.
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