Predicting Decline In Agrammatism Apraxia Of Speech And Parkinsonism In Agrammatic Primary Progressive Aphasia

OBJECTIVE: To determine whether baseline neuroimaging predicts future rate of clinical decline in patients with agrammatic primary progressive aphasia (agPPA) or progressive apraxia of speech (PAOS).BACKGROUND: Agrammatism and apraxia of speech are characteristic of both agPPA and PAOS. Rate of decline in these features is variable making prognosis difficult. These patients also often develop Parkinsonism, with a subset progressing rapidly into a Parkinsonian disorder. Identifying biomarkers that can help predict rate of clinical decline will be critical to improve prognostic estimates.DESIGN/METHODS: Thirty-eight patients with agPPA or PAOS underwent baseline speech and language and neurological assessments, and MRI, and then returned two years later. Agrammatism was measured using the Western Aphasia Battery Aphasia Quotient (WAB-AQ), apraxia of speech was measured using the Apraxia of Speech Rating Scale (ASRS), and Parkinsonism was measured using the Unified Parkinson’s Disease Rating Scale-3 (UPDRS-3). Baseline regional grey matter volumes were calculated. Mixed effects models were used to determine the degree to which baseline volumes predict future rate of decline in the clinical measures.RESULTS: Mean annual rate of decline was -4 points (95[percnt] CI: -14 to 5) for WAB-AQ, 3 points (-3 to 9) for ASRS and 8 points (-9 to 26) for UPDRS-3. Small baseline volumes of supplementary motor area, premotor, precentral and paracentral cortex were associated with increased rates of decline on the UPDRS-3. Large volumes (i.e. relative sparing) of Broca9s area was associated with increased rates of decline on the ASRS and large paracentral volumes were associated with increased rates of decline on the WAB-AQ.CONCLUSIONS: Neuroimaging measures have potential to be useful biomarkers of future clinical decline in these patients, particularly with atrophic premotor and motor regions predicting decline in Parkinsonism.Study supported by: R01-DC12519 Disclosure: Dr. Whitwell has nothing to disclose. Dr. Weigand has nothing to disclose. Dr. Duffy has nothing to disclose. Dr. Strand has nothing to disclose. Dr. Machulda has nothing to disclose. Dr. Clark has nothing to disclose. Dr. Senjem has nothing to disclose. Dr. Jack has received personal compensation for activities with Janssen Research u0026 Development, LLC by providing consulting services. Dr. Jack has received research support from the National Institutes of Health (R01-AG011378, RO1-AG041851, RO1-AG037551. Dr. Josephs has nothing to disclose.