Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
 12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:An unusual cause of dementia: essential diagnostic elements of corticobasal degeneration-a case report and review of the literature. 2178

Corticobasal degeneration (CBD) is a neurodegenerative disease characterised by linear progression, asymmetrical and extrapyramidal symptoms such as rigor and dystonia, as well as by variable cortical symptoms including apraxia, cortical sensory deficits, the alien limb phenomenon and myoclonism of the reflexes. Pathological changes of CBD consist of characteristic taupathology in the gray and white matter. However, there are also patients with neurodegenerative diseases with a different underlying pathology that nevertheless appear clinically as CBD. For that reason, the term corticobasal syndrome (CBS) is commonly used to describe the clinical features, whereas the term CBD is reserved for the pathological entity. Moreover, patients with the typical pathology of CBD can present clinical signs consistent with a clinical diagnosis of Alzheimer's disease (AD) or progressive supranuclear palsy (PSP). We demonstrate this clinico-pathological heterogeneity by presenting two illustrative case reports. The first patient developed the typical clinical symptoms of progressive supranuclear palsy, while exhibiting pathologically CBD. The second patient showed clinical signs of CBS, although pathologically she was diagnosed with Alzheimer's disease. These exemplary cases underscore the need to distinguish carefully between the clinical syndrome of CBS and the pathologically defined entity of CBD.
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PMID:[The diagnostic challenge of corticobasal degeneration: distinction between clinical syndrome and pathology]. 2200 74

Corticobasal degeneration is a degenerative disease characterized by asymmetric brain atrophy and clinically by asymmetric onset of an akinetic-rigid syndrome with apraxia, dysarthria and dysphagia. Diagnosis must be confirmed by autopsy. We have investigated the ability of MRI to detect asymmetric atrophy to support the clinical diagnosis and permit differential diagnosis against other degenerative disorders. Ten patients with clinical suspicion of corticobasal degeneration were studied by brain MRI, and the images were reviewed with the side of greater clinical involvement unknown to the reviewer. The original reports of MR scans were also reviewed. MRI demonstrates that cortical atrophy is asymmetric and more marked in the posterior frontal and mainly in the parietal regions on the side contralateral to the clinical symptoms. Asymmetry was rarely detected on the first reading. Our review of MRI findings demonstrates that it is possible to detect asymmetrical parietal atrophy, thus supporting the clinical diagnosis of corticobasal degeneration. It is essential to be aware of the disease and alert for asymmetries in order to discern the more involved side. No abnormalities were detected in the basal ganglia.
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PMID:MRI in corticobasal degeneration. 2428 81


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