Phenotypic variability in ALS-FTD and effect on survival
| Autor: | John R. Hodges, Matthew C. Kiernan, Emma Devenney, Olivier Piguet, Rebekah M. Ahmed, Cherie Strikwerda-Brown |
|---|---|
| Rok vydání: | 2020 |
| Předmět: |
0301 basic medicine
education.field_of_study medicine.medical_specialty medicine.diagnostic_test business.industry Population Magnetic resonance imaging Cognition Audiology medicine.disease 03 medical and health sciences 030104 developmental biology 0302 clinical medicine Atrophy Neuroimaging medicine Dementia Neurology (clinical) Presentation (obstetrics) education business 030217 neurology & neurosurgery Survival analysis |
| Zdroj: | Neurology. 94:e2005-e2013 |
| ISSN: | 1526-632X 0028-3878 |
| DOI: | 10.1212/wnl.0000000000009398 |
| Popis: | ObjectiveTo determine if survival and cognitive profile is affected by initial presentation in amyotrophic lateral sclerosis–frontotemporal dementia (ALS-FTD) (motor vs cognitive), we compared survival patterns in ALS-FTD based on initial phenotypic presentation and their cognitive profile compared to behavioral variant FTD (bvFTD).MethodsCognitive/behavioral profiles were examined in 98 patients (59 ALS-FTD and 39 bvFTD). The initial presentation of ALS-FTD was categorized into either motor or cognitive. Survival was calculated from initial symptom onset. MRI brain atrophy patterns were examined using a validated visual rating scale.ResultsIn the ALS-FTD group, 41 (69%) patients were categorized as having an initial cognitive presentation and 18 (31%) a motor presentation. Patients with motor presentation experienced a significantly shorter median survival of 2.7 years compared to 4.4 years (p < 0.001) in those with a cognitive presentation. No differences between motor vs cognitive onset ALS-FTD were found on cognitive testing. When compared to bvFTD, ALS-FTD–cognitive presentation was characterized by reduced language function (p < 0.001), verbal fluency (p = 0.001), and naming (p = 0.007). Both motor and cognitive onset ALS-FTD showed reduced emotion processing (p = 0.01) and exhibited greater motor cortex and dorsal lateral prefrontal cortex atrophy than bvFTD. Increased motor cortex atrophy was associated with 1.5-fold reduction in survival.ConclusionsInitial motor presentation in ALS-FTD leads to faster progression than in those with a cognitive presentation, despite similar overall cognitive deficits. These findings suggest that disease progression in ALS-FTD may be critically linked to physiologic and motor changes. |
| Databáze: | OpenAIRE |
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