An Unusual Cause of Dementia: Essential Diagnostic Elements of Corticobasal Degeneration—A Case Report and Review of the LiteratureReport as inadecuate

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International Journal of Alzheimer-s DiseaseVolume 2011 2011, Article ID 536141, 6 pages

Case ReportDepartment of Neurology, “Campus Biomedico” University, Via Álvaro del Portillo, 21-00128, Rome, Italy

Received 15 January 2011; Revised 21 April 2011; Accepted 18 May 2011

Academic Editor: Florinda Ferreri

Copyright © 2011 F. Mastrolilli et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Corticobasal degeneration CBD is an uncommon, sporadic, neurodegenerative disorder of mid- to late-adult life. We describe a further example of the pathologic heterogeneity of this condition. A 71-year-old woman initially presented dysarthria, clumsiness, progressive asymmetric bradykinesia, and rigidity in left arm. Rigidity gradually involved ipsilateral leg; postural instability with falls, blepharospasm, and dysphagia subsequently developed. She has been previously diagnosed as unresponsive Parkinson-s Disease. At our clinical examination, she presented left upper-arm-fixed-dystonia, spasticity in left lower limb and pyramidal signs Babinski and Hoffmann. Brain MRI showed asymmetric cortical atrophy in the right frontotemporal cortex. Neuropsychological examination showed an impairment in visuospatial functioning, frontal-executive dysfunction, and hemineglect. This case demonstrates that association of asymmetrical focal cortical and subcortical features remains the clinical hallmark of this condition. There are no absolute markers for the clinical diagnosis that is complicated by the variability of presentation involving also cognitive symptoms that are reviewed in the paper. Despite the difficulty of diagnosing CBD, somatosensory evoked potentials, motor evoked potentials, long latency reflexes, and correlations between results on electroencephalography EEG and electromyography EMG provide further support for a CBD diagnosis. These techniques are also used to identify neurophysiological correlates of the neurological signs of the disease.

Author: F. Mastrolilli, A. Benvenga, L. Di Biase, F. Giambattistelli, L. Trotta, G. Salomone, L. Quintiliani, D. Landi, J. M. Melg



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