This is an abstract of a recently published article giving a case report of one woman with a clinical diagnosis of CBD. The symptoms and signs all point towards CBD — 71 years old, dysarthria, clumsiness, progressive asymmetric bradykinesia, rigidity in left arm, postural instability with falls, blepharospasm, dysphagia, dystonia in right arm, pyramidal signs (Babinski and Hoffmann), asymmetric cortical atrophy on MRI, visuospatial impairment, frontal-executive dysfunction, and hemineglect. The title of the article refers to “an unusual case of dementia,” but we aren’t told in the abstract why this case is unusual or given any details of the dementia (except we are told about the frontal-executive dysfunction).
Presumably the woman is still alive or she has died and didn’t donate her brain because there doesn’t seem to be pathologic confirmation of the diagnosis.
Robin
International Journal of Alzheimers Disease. 2011;2011:536141. Epub 2011 Jul 12.
An unusual cause of dementia: essential diagnostic elements of corticobasal degeneration-a case report and review of the literature.
Mastrolilli F, Benvenga A, Di Biase L, Giambattistelli F, Trotta L, Salomone G, Quintiliani L, Landi D, Melgari JM, Vernieri F.
Department of Neurology, “Campus Biomedico” University, Rome, Italy.
Abstract
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.
PubMedID#: 21785700 (see pubmed.gov for this abstract only)