Highlights
- •In MSA autonomic dysfunction within the first 3 years from the disease onset is associated with rapid progression to death.
- •In MSA urinary impairment at baseline may help to select ‘fast progressors’ in terms of neuronal loss.
- •Urinary impairment should be recognized as a key target for disease modifying therapeutic interventions in MSA.
Abstract
Introduction
MSA is an adult-onset, sporadic, progressive parkinsonian syndrome characterised by
the presence of akinesia, cerebellar dysfunction, autonomic failure and pyramidal
signs. Annualized-whole-brain atrophy rate (a-WBAR) is an informative way to quantify
disease progression. In this longitudinal work we investigate the correlations of
a-WBAR with clinical scales for motor impairment, autonomic disability and cognitive
decline in MSA and explore how atrophy progresses within the brain.
Method
Fourty-one MSA patients were studied using Structural Imaging Evaluation with Normalization
of Atrophy (SIENA). SIENA is an MRI-based algorithm that quantifies brain tissue volume.
Clinical parameters were explored using the 18-item Movement Disorder Society-sponsored
revision of the Unified Parkinson's Disease Rating Scale, the Hoehn and Yahr Scale,
the Frontal Assessment Battery and the Natural History and Neuroprotection in Parkinson
Plus Syndromes scale (sub-items for orthostatic and urinary functions).
Results
The mean (±SD) age was 60.4 years ± 7.7 and a-WBAR was 1.65% ± 0.9. Demographics and
clinical ratings at the time of the first scan were non-significantly associated with
a-WBAR. The only exception was the baseline urinary score with a weak but significant
association (R2 = 0.15, p = 0.04). Progression of grey matter atrophy was detected in the left superior temporal
gyrus, right middle frontal gyrus, right frontopolar region and midbrain.
Conclusion
Urinary impairment at baseline may help to identify ‘fast progressors’ in terms of
neuronal loss, particularly in the frontal and temporal lobes. Thus, urinary impairment
should be recognized as a key target for disease modifying therapeutic interventions
in MSA.
Keywords
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Article info
Publication history
Published online: December 13, 2018
Accepted:
December 13,
2018
Received in revised form:
November 11,
2018
Received:
June 4,
2018
Identification
Copyright
© 2018 Elsevier B.V. All rights reserved.