Neuropsychiatric Features of Corticobasal Degeneration
J Neurol Neurosurg Psychiatry 1998;65:717–721 717 J Neurol Neurosurg Psychiatry: first published as 10.1136/jnnp.65.5.717 on 1 November 1998. Downloaded from Neuropsychiatric features of corticobasal degeneration Irene Litvan, JeVrey L Cummings, Michael Mega Abstract Corticobasal degeneration (CBD) is a neuro- Objective—To characterise the neuropsy- degenerative disorder characterised clinically chiatric symptoms of patients with corti- by steadily progressive motor (for example, cobasal degeneration (CBD). dystonia, asymmetric parkinsonism not ben- Methods—The neuropsychiatric inven- efitting from levodopa therapy, myoclonus, tory (NPI), a tool with established validity balance disturbances, and pyramidal signs) and and reliability, was administered to 15 cognitive (for example, ideomotor apraxia, patients with CBD (mean (SEM), age 67.9 alien hand syndrome, aphasia, visual and (2) years); 34 patients with progressive sensory neglect) disturbances presenting after supranuclear palsy (PSP) (66.6 (1.2) the age of 40.12Neuropathologically, it features years); and 25 controls (70 (0.8) years), circumscribed parietal or frontoparietal atro- matched for age and education. Both phy, severe cortical neuronal loss, and intense patient groups had similar duration of astrogliosis, spongiosis, swollen and achro- symptoms and mini mental state exam- matic neurons (ballooned cells), neuropil ination scores. Semantic fluency and threads, and occasionally, neurofibrillary tan- motor impairment were also assessed. gles. In addition, basophilic argyrophilic and Results—Patients with CBD exhibited tau protein positive inclusions are found in the depression (73%), apathy (40%), irritabil- neurons of the substantia nigra and basal gan- ity (20%), and agitation (20%) but less glia (subthalamic nucleus, striatum, and palli- often had anxiety, disinhibition, delusions, dum), and also may be present along the den- or aberrant motor behaviour (for exam- tatorubrothalamic tracts.
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