title: Pathology and sensitivity of current clinical criteria in corticobasal syndrome.
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 ± standard deviation, 67.9 ± 9.3 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.
authors.affiliation: Department of Neurology, Brain Research Institute, Niigata University, Niigata, Japan.
authors.forename: Haruka
authors.initials: H
authors.lastname: Ouchi
authors.forename: Yasuko
authors.initials: Y
authors.lastname: Toyoshima
authors.forename: Mari
authors.initials: M
authors.lastname: Tada
authors.forename: Mutsuo
authors.initials: M
authors.lastname: Oyake
authors.forename: Izumi
authors.initials: I
authors.lastname: Aida
authors.forename: Itsuro
authors.initials: I
authors.lastname: Tomita
authors.forename: Akira
authors.initials: A
authors.lastname: Satoh
authors.forename: Mitsuhiro
authors.initials: M
authors.lastname: Tsujihata
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Takahashi
authors.forename: Masatoyo
authors.initials: M
authors.lastname: Nishizawa
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Shimohata
mesh_terms: D000368:Aged; D000544:Alzheimer Disease / Q000209:etiology; D001479:Basal Ganglia / Q000473:pathology*; D001927:Brain Diseases / Q000150:complications* / Q000175:diagnosis*; D002540:Cerebral Cortex / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013494:Supranuclear Palsy, Progressive / Q000209:etiology; D024801:Tauopathies / Q000209:etiology
keywords: corticobasal degeneration; corticobasal syndrome; diagnostic criteria