This is a newly-published Mayo Rochester study of 24 patients with a clinical diagnosis of CBS (corticobasal syndrome) who had undergone MRI during life and donated their brains upon death. These 24 patients had these pathologic diagnoses:
7 had CBD
6 had PSP
6 had AD (Alzheimer’s Disease)
5 had FTLD with TDP-43
29% were diagnosed accurately during life. 54% were diagnosed accurately during life if you include the PSP diagnoses. (As PSP and CBS/CBD are treated similarly and are both tauopathies, I’m willing to consider PSP a correct diagnosis. Many of you will not agree.)
Indeed, in our local support group, several diagnosed while alive with CBS/CBD turned out to have had PSP. And there are many on the CBD-related Yahoo!Group who report that their family members were diagnosed during life with CBS/CBD but upon death with AD.
The researchers then looked at the previous MRI scans to “determine whether patterns of atrophy on imaging could be useful to help predict underlying pathology in CBS.” They found:
“Widespread atrophy points toward a pathologic diagnosis of FTLD-TDP or AD, with frontotemporal loss suggesting FTLD-TDP and temporoparietal loss suggesting AD. On the contrary, more focal atrophy predominantly involving the premotor and supplemental motor area suggests CBD or PSP pathology.”
It’s a nice study. Wish it could’ve been with more than 24 patients.
Robin
Neurology. 2010 Nov 23;75(21):1879-87.
Imaging correlates of pathology in corticobasal syndrome.
Whitwell JL, Jack CR Jr, Boeve BF, Parisi JE, Ahlskog JE, Drubach DA, Senjem ML, Knopman DS, Petersen RC, Dickson DW, Josephs KA.
Department of Radiology, 200 1st Street SW, Rochester, MN.
Abstract
BACKGROUND: Corticobasal syndrome (CBS) can be associated with different underlying pathologies that are difficult to predict based on clinical presentation. The aim of this study was to determine whether patterns of atrophy on imaging could be useful to help predict underlying pathology in CBS.
METHODS: This was a case-control study of 24 patients with CBS who had undergone MRI during life and came to autopsy. Pathologic diagnoses included frontotemporal lobar degeneration (FTLD) with TDP-43 immunoreactivity in 5 (CBS-TDP), Alzheimer disease (AD) in 6 (CBS-AD), corticobasal degeneration in 7 (CBS-CBD), and progressive supranuclear palsy in 6 (CBS-PSP). Voxel-based morphometry and atlas-based parcellation were used to assess atrophy across the CBS groups and compared to 24 age- and gender-matched controls.
RESULTS: All CBS pathologic groups showed gray matter loss in premotor cortices, supplemental motor area, and insula on imaging. However, CBS-TDP and CBS-AD showed more widespread patterns of loss, with frontotemporal loss observed in CBS-TDP and temporoparietal loss observed in CBS-AD. CBS-TDP showed significantly greater loss in prefrontal cortex than the other groups, whereas CBS-AD showed significantly greater loss in parietal lobe than the other groups. The focus of loss was similar in CBS-CBD and CBS-PSP, although more severe in CBS-CBD.
CONCLUSIONS: Imaging patterns of atrophy in CBS vary according to pathologic diagnosis. Widespread atrophy points toward a pathologic diagnosis of FTLD-TDP or AD, with frontotemporal loss suggesting FTLD-TDP and temporoparietal loss suggesting AD. On the contrary, more focal atrophy predominantly involving the premotor and supplemental motor area suggests CBD or PSP pathology.
PubMed ID#: 21098403