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From the Neuroepidemiology Branch (Dr. Litvan), National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD; the Federation de Neurologie and INSERM U 289 (Drs. Agid and Brandel), Hopital de la Salpetriere, Paris, France; Department of Neurology (Dr. Goetz), Rush Medical College, Chicago, IL; Department of Neurology (Drs. Jankovic and Lai), Baylor College of Medicine, Houston, TX; the Parkinson's Disease Society Brain Tissue Bank (Drs. Wenning and Pearce), Institute of Neurology, London, UK; Department of Neurology (Dr. Ray-Chaudhuri), Institute of Psychiatry, London, UK; the Raymond Escourolle Neuropathology Laboratory (Dr. Verny), INSERM U 360, Hopital de la Salpetriere, Paris, France; Department of Neuropathology (Dr. McKee), Massachusetts General Hospital, Boston, MA; the Ludwig Boltzmann Institute of Clinical Neurobiology (Dr. Jellinger), Vienna, Austria; and Division of Epidemiology and Research Studies (Dr. Bartko), National Institute of Mental Health, Bethesda, MD.
Presented in part at the Movement Disorder Society Symposium on Cortical-Basal Ganglionic Degeneration (CBGD) and Its Relationship to Other Asymmetrical Cortical Degeneration Syndromes (ACDs), Washington, DC, October 1995.
Received March 21, 1996. Accepted in final form June 14, 1996.
Address correspondence and reprint requests to Dr. Irene Litvan, Federal Building, Room 714, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD 20892-9130.
The accuracy of the clinical diagnosis of corticobasal degeneration (CBD) is unknown.To determine its diagnostic accuracy, we presented 105 cases with known neuropathologic diagnoses, including CBD (n = 10), progressive supranuclear palsy (PSP, n = 24), Parkinson's disease (n = 15), diffuse Lewy body disease (n = 14), multiple system atrophy (n = 16), postencephalitic parkinsonism (n = 7), Pick's disease (n = 7), Creutzfeldt-Jakob disease (n = 4), Alzheimer's disease (n = 4), vascular parkinsonism (n = 3), and Whipple's disease (n = 1), as clinical vignettes to six neurologists unaware of the autopsy findings. Reliability was measured with the kappa statistics. The neurologists' clinical diagnoses were compared with clinicopathologic diagnoses for sensitivity, specificity, and positive predictive values at first and last clinic visits. The group reliability for the diagnosis of CBD significantly improved from moderate for the first visit (mean = 34 months after onset) to substantial for the last (68 months after onset). For the first visit, mean sensitivity for CBD was low (35%), but specificity was near-perfect (99.6%). For the last visit, mean sensitivity minimally increased (48.3%), and specificity remained stable. False-negative misdiagnoses mainly occurred with PSP. False-positive diagnoses were rare. The extremely low sensitivity of the clinical diagnosis of CBD suggests that this disorder is markedly underdiagnosed. Although the validity of the clinical diagnosis might have been improved if neurologists could have examined these patients, more important is that this disorder was misdiagnosed by the primary neurologists. In our data set, the best predictors for the diagnosis of CBD included limb dystonia, ideomotor apraxia, myoclonus, and asymmetric akinetic-rigid syndrome with late onset of gait or balance disturbances.
NEUROLOGY 1997;48: 119-125
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