2013.12.09
Pathology and sensitivity of current clinical criteria in corticobasal syndrome
（Mov Disord. 2014 Feb;29(2):238-44. doi: 10.1002/mds.25746. Epub 2013 Nov 20.）
Ouchi H1, Toyoshima Y2, Tada M2, Oyake M3, Aida I4, Tomita I5, Satoh A5, Tsujihata M5, Takahashi H2, Nishizawa M1, Shimohata T1.
1Department of Neurology, Brain Research Institute, Niigata University
2Department of Pathology, Brain Research Institute, Niigata University
3Department of Neurology, Nagaoka Red Cross Hospital, Nagaoka
4Department of Neurology, Niigata National Hospital, National Hospital Organization, Kashiwazaki
5Department of Neurology, Nagasaki Kita Hospital, Nagasaki
Abstract
The aim of this study was to investigate corticobasal syndrome with respect to underlying pathologies, the ability of current clinical criteria to detect early stages of disease, and symptoms and signs predicting background pathologies. We retrospectively analyzed the clinicopathological findings from patients with corticobasal syndrome. We also analyzed whether those findings fulfilled the diagnostic criteria for corticobasal degeneration (CBD). Finally, we investigated characteristic clinical features that are specific to each background pathology. Of 10 consecutive autopsied patients who had corticobasal syndrome (mean age 6 standard deviation, 67.9 69.3 years; male:female ratio, 6:4), three had corticobasal degeneration pathology, three had progressive supranuclear palsy, three had Alzheimer's disease, and one had atypical four-repeat tauopathy. Nine patients fulfilled Mayo criteria, and all 10 patients fulfilled modified Cambridge criteria at the later stage, but only two patients fulfilled either clinical criteria within 2 years of disease onset. Five patients fulfilled the clinical criteria for possible CBD (p-CBD), and one patient fulfilled the clinical research criteria for probable sporadic CBD (cr-CBD) at the later stage. Only two patients fulfilled the criteria for either p-CBD or cr-CBD within 2 years of disease onset. Although we could not find any predictive characteristic clinical features that were specific to CBD pathology, only patients with progressive supranuclear palsy developed apraxia of eyelid opening and cerebellar ataxia. Myoclonus and memory impairment, especially if they appear at an early stage of the disease, may predict Alzheimer's disease pathology. Sensitivity of the available clinical criteria for corticobasal syndrome was poor within 2 years of disease onset.
