Table 6.

Summary of clinical changes in longitudinal studies of patients presenting with PPAOS.

Reference	Sample size and clinical measures	Key Findings
Group-level changes
Josephs et al. (2014)	• 13 patients with PPAOS • Median of 4.5 years post symptom onset at visit 1 • 2-3 year follow up • ASRS	• ASRS = 16 at baseline (scores above about 8-9 considered consistent with AOS) and 26.5 at follow-up • Annualized change: 2.5 points ( practical meaning difficult to interpret)
Whitwell et al. (2017)	• 20 patients with PPAOS • Median of 3.5 years post symptom onset at visit 1 • 2-year follow up • AOS severity rating and MSD rating	• 8 patients developed aphasia at follow-up; 60% of patients still without aphasia at about 5 to 6 years post symptom onset • Median AOS severity rating = 1 (mild) to 1.5 (mild-moderate) at initial visit, regardless of aphasia; annualized rate of change = 0.5 points for entire group • Median MSD rating at initial assessment = 7; annualized rate of change = 0.7 points and 0.8 points, regardless of aphasia
Utianski et al. (2020)	• 13 patients with PPAOS • Median of 4 years post symptom onset at visit 1 • 1-year follow up • ASRS and AOS severity rating	• 8/13 patients (62%) declined on both measures
Individual-level changes
Duffy et al. (2015)	• 2 patients with PPAOS  ο Case 1: seen at 5 and 7.5 years post-onset  ο Case 2: seen at 2 and 4 years post onset • AOS severity rating, MSD severity rating, ASRS, AES, and temporal acoustics	• Case 1: AOS severity changed from moderate (2) to severe (4); MSD severity from 6 to 3; ASRS from 21 to 35, and AES from 52% to 63% • Case 2: AOS severity changed from mild (1) to moderate (2), MSD severity from 7 to 6; ASRS from 18 to 21; AES from 7% to 13% • Syllables per second for three multisyllabic words, a sentence, and speech AMRs and SMRs was abnormal at first testing for both cases and further reduced at second visit for both patients
Utianski, Duffy, Clark, Strand, Boland, et al. (2018)	• 4 patients with PPAOS • 1.5, 2, 4, and 10 years post symptom onset at visit 1 • Followed yearly for 5-6 years • AOS severity rating, MSD severity rating, ASRS, AES, and temporal acoustics	• AOS severity worsened from mild to marked or severe in all patients • MSD severity dropped from 6, 7, or 8 to 1 or 2 over time • ASRS scores increased from 33 to 48, 15 to 30, 11 to 34, and 1 to 29 between first and last measureable assessment • AES changed from 68% to 86%, 13% to 41%, 5% to 47%, and 11% to 78% between first and last measurable assessment • Acoustically measured syllable per second rate for the word “catastrophe” abnormally slow for all patients at initial assessment and became slower over time until they were no longer able to produce four syllable word • All patients using AAC (writing tablet, iPad with speaking app) by 7 to 10 years post symptom onset
Tetzloff et al. (2018)	• 1 patient with PPAOS • Followed 5, 8, 9, and 10 years post symptom onset • MSD severity rating, ASRS	• MSD rating scale scores of 6, 3, 3, and 1 across visits • ASRS scores of 20, 38, 39, and untestable at last visit • Dysarthria, aphasia, and parkinsonism emerged by 8 years post onset • Diagnostic criteria for CBS met by 9 years • CBD pathology at autopsy