The Italian researchers show that “albeit the overall pattern of brain atrophy associated with PSP appears remarkably consistent across the spectrum of clinical features recorded in life, major anatomical differences between these two conditions [Richardson’s syndrome and PSP-parkinsonism — the two main types of PSP] do exist. Such a different topographical distribution of tissue damage may account for the clinical differences between PSP-RS and PSP-P.”
Of course autopsy confirmation of these diagnoses (RS and PSP-P) are not available from the 20 patient cases in the study.
What would be the clinical relevance of determining which type of PSP someone has? Survival time is different, and this may be of interest to families and patients. Response to levodopa is different. The expectation would be for someone with RS to experience cognitive impairment, falls, and speech impairment (dysarthria) early on, so compensatory steps could be taken by families for these symptoms and training could be found on how to deal with these symptoms. Does anyone else have thoughts about this?
Robin
European Journal of Neuroscience. 2010 Jun 30. [Epub ahead of print]
The in vivo distribution of brain tissue loss in Richardson’s syndrome and PSP-parkinsonism: a VBM-DARTEL study.
Agosta F, Kostic VS, Galantucci S, Mesaroš S, Svetel M, Pagani E, Stefanova E, Filippi M.
Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, Scientific Institute and University Hospital San Raffaele, Milan, Italy.
Abstract
In this study, we wished to test, using magnetic resonance imaging and voxel-based morphometry (VBM), whether specific cortical and subcortical patterns of brain grey (GM) and white matter (WM) tissue loss can be detected in patients with Richardson’s syndrome (PSP-RS) and progressive supranuclear palsy-parkinsonism (PSP-P), and possibly account for their clinical heterogeneity.
Twenty patients with PSP, classified as PSP-RS (10 patients) or PSP-P (10 patients), and 24 healthy controls were studied. The Statistical Parametric Mapping (SPM5) and the Diffeomorphic Anatomical Registration using Exponentiated Lie algebra method were used to perform a VBM analysis.
Compared with controls, both patient groups showed GM loss in the central midbrain, cerebellar lobes, caudate nuclei, frontotemporal cortices and right hippocampus.
WM loss was detected in both conditions in the midbrain, left superior cerebellar peduncle, internal capsulae, and left premotor and bilateral prefrontal regions.
Compared with PSP-P, patients with PSP-RS showed additional regions of GM loss in the midbrain, left cerebellar lobe and dentate nuclei. PSP-RS was also associated with a more severe WM loss in the midbrain, internal capsulae, and orbitofrontal, prefrontal and precentral/premotor regions, bilaterally.
Patients with PSP-P showed a more pronounced GM loss only in the frontal cortex, bilaterally.
This study shows that, albeit the overall pattern of brain atrophy associated with PSP appears remarkably consistent across the spectrum of clinical features recorded in life, major anatomical differences between these two conditions do exist. Such a different topographical distribution of tissue damage may account for the clinical differences between PSP-RS and PSP-P.
PubMed ID#: 20597976 (see pubmed.gov for this abstract only)