Table 2.
Baseline demographic and clinical features for the five +AOS cases with FTLD-TDP, PiD, and AGD pathology.
| FTLD-TDP A | FTLD-TDP A | FTLD-TDP B + AGD | PiD | PiD | |
|---|---|---|---|---|---|
| Demographics and genetic data | |||||
| Onset to death, years | 5 | 5 | 2 | 8 | 6 |
| Onset to baseline, years | 2 | 3 | 2 | 4 | 2 |
| FTD mutations | GRN | GRN | None | None | None |
| APOE genotype | 3/4 | 3/3 | 3/3 | 3/3 | 2/3 |
| H1 haplotype | H1/H1 | H1/H2 | H1/H1 | H1/H1 | H1/H2 |
| Family history in 1st or 2nd degree relative | None | None | None | Present | Present |
| TMEM106B CC/CG/GG | CG | CG | CC | CC | GG |
| Neurological data | |||||
| Presenting symptoms | (Executive dysfunction, parkinsonism) + AOS | (Mild behavioral changes, aphasia) + AOS | (Dysarthria, dysphagia, face/tongue weakness anomia) + AOS | (Mild behavioral changes, aphasia) + AOS | (Obsessive compulsive behaviors) + AOS |
| Final diagnosis before death | CBS | Probable bvFTD | Unclassified | Probable bvFTD | Possible bvFTD |
| MMSE, /30 | 30 | 13 | 24 | 29 | 27 |
| MoCA, /30 | 28 | 17 | 24 | 29 | 22 |
| FAB, /18 | 18 | 8 | 15 | 18 | 15 |
| FBI, /72, 0 = best | 4 | 21 | 8 | 28 | 7 |
| NPI-Q, /36 | 0 | 7 | 7 | 2 | 5 |
| MDS-UPDRS III, /132, 0 = best | 9 | 5 | 24 | 7 | 11 |
| WAB Praxis, /60 | 60 | 49 | 57 | 57 | 58 |
| PSIS, /5, 0 = best | 0 | 1 | 0 | 0 | 0 |
| Speech and language data | |||||
| AOS subtype | Phonetic | Phonetic | Phonetic | Phonetic | Phonetic |
| Aphasia present | No | Yes | Yes | Yes | No |
| Aphasia severity /4 | 0 | 2 | 1 | 1 | 0 |
| WAB Aphasia Quotient, /100 | 97 | 78 | 95 | 96 | 96 |
| ASRS Total Score V3 | 10 | 11 | 10 | 14 | 6 |
| ASRS Phonetic subscore | 6 | 5 | 4 | 7 | 4 |
| ASRS Prosodic subscore | 0 | 2 | 2 | 2 | 0 |
| MSD Severity Scale, /10 | 6 | 5 | 7 | 6 | 9 |
| BNT, /15 | 14 | 13 | 10 | 13 | 14 |
| WAB fluency, /10 | 9 | 5 | 9 | 10 | 9 |
| Letter fluency (FAS) | 46 | 12 | 14 | 21 | 16 |
| WAB animal fluency | 20 | 13 | 11 | 20 | 13 |
| Token Test, /22 | 19 | 4 | 19 | 21 | 17 |
| NAT, /10 | 9 | 6 | 10 | 8 | 9 |
| PPT word-word, /52 | 52 | 41 | 50 | 49 | 51 |
| Dysarthria Present, N % | No | No | Yes | No | No |
| Dysarthria Severity, /4 | 0 | 0 | 1 | 0 | 0 |
| NVOA, /32, 32 = normal | 31 | 17 | 31 | 14 | 21 |
| Neuropsychological data | |||||
| TMT A MOANS | 11 | 5 | 13 | 11 | 7 |
| TMT B MOANS | 7 | 3 | 9 | 12 | 3 |
| Sorting Test SS | 12 | 0 | 11 | 13 | 3 |
| WMS-III VR I SS | 7 | 5 | 9 | 8 | 2 |
| WMS-III VR II SS | 9 | 9 | 8 | 13 | 5 |
| VOSP letters, /20 | 20 | 19 | 20 | 20 | 19 |
| VOSP cubes, /10 | 10 | 5 | 10 | 8 | 6 |
MOANS and SS have a mean of 10 with a standard deviation of 3 in normal healthy individuals.
APOE apolipoprotein, GRN progranulin, MAPT microtubule associated protein tau, MMSE Mini Mental State Examination, MoCA Montreal Cognitive Assessment, FAB Frontal Assessment Battery, FBI Frontal Behavioral Inventory, NPI-Q short questionnaire version of the Neuropsychiatric Inventory, MDS-UPDRS III Movement Disorder Society sponsored revision of the Unified Parkinson’s Disease Rating Scale part III, WAB Western Aphasia Battery, PSIS Progressive supranuclear palsy Saccadic Impairment Scale, AOS apraxia of speech, ASRS Apraxia of Speech Rating Scale, MSD Motor Speech Disorder, BNT Boston Naming Test, NAT Northwestern Anagram Test, PPT Pyramids and Palm Trees test, NVOA nonverbal oral apraxia, SS scaled score, TMT Trail Making Test, WMS-III VR Wechsler Memory Scale-III Visual Reproduction, MOANS Mayo Older American Normative Studies, VOSP Visual Object and Space Perception Battery.