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Query: UMLS:C0030567 (Parkinson's disease)
 63,064 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A progressive parkinsonian disorder predicts pathology in the substantia nigra and possibly elsewhere in the basal ganglia. Parkinson's disease is a manifestation of Lewy body disease, which is characterized by the association between Lewy bodies and cell degeneration in specific neuronal populations. Striatonigral degeneration is part of multiple system atrophy and is characterized by striatal and nigral degeneration without neuronal inclusion bodies, but glial inclusions have been described. Steele-Richardson-Olszewski disease is characterized by the globose neurofibrillary tangle and predominant brain stem pathology. Corticobasal degeneration shows similar midbrain pathology and a round, filamentous inclusion in the substantia nigra, not unlike the globose tangle, but there is also focal frontoparietal cortical atrophy. The combination of the distribution of degeneration and nerve cell morphology identify apparently distinct disorders, but most of the neuronal inclusions are not disease specific.
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PMID:Neuropathology of Parkinson's disease and related syndromes. 158 79

Corticomotoneuronal excitability was examined in 7 patients with dopa-nonresponsive progressive hemiparkinsonism (DNRHP) and 10 with dopa-responsive hemiparkinsonism (Parkinson's disease: PD), as well as in 10 normal subjects, by measuring change in motor evoked potentials (MEPs) using transcranial magnetic stimulation of the motor cortex after peripheral nerve stimulation. Corticobasal degeneration (CBD) was suspected clinically for the progressive dopa-nonresponsive hemiparkinsonism. Conditioning stimulation of the median nerve on the rigid side greatly increased MEP size (500-1300%) in 4 DNRHP patients as compared to the increase for the normal subjects (140-380%) at conditioning test (C-T) intervals of 40-80 ms, but stimulation on the contralateral side did not. There was no abnormal MEP facilitation on the rigid side in 10 PD patients. None of the patients with CBD had an increase in somatosensory evoked potential (SEP) size, and no patient showed hyperexcitability in SEP-recovery during C-T intervals at which there was abnormal MEP facilitation. These results indicate that there is an exaggerated effect of afferent input on corticomotoneuronal excitability in some patients with DNRHP.
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PMID:Increased corticomotoneuronal excitability after peripheral nerve stimulation in dopa-nonresponsive hemiparkinsonism. 775 42

Corticobasal degeneration (CBD) is a rare syndrome characterised by an asymmetrical rigidity with localised cortical signs, particularly apraxia. Using positron emission tomography, abnormal patterns of cortical metabolism have recently been shown. We have studied patterns of regional cerebral blood flow (rCBF) using single photon emission tomography, with the tracer 99Technetium hexamethylpropylenamine (HMPAO), in subjects with CBD. In subjects with CBD, compared with 12 age-matched normal controls, in the clinically more affected hemisphere a characteristic pattern was found with significant reductions in HMPAO uptake in the posterior frontal cortex (by 11.5%), and in the superior, inferior, anterior, and posterior parietal cortex (by 12.2, 12.9, 12.9, and 9.7, respectively). Reduced uptake was also found in the caudate (9.3%), putamen (9.7%), and thalamus (8.6%). In contrast, HMPAO uptake in the temporal and occipital cortex was normal. In comparison with 12 Parkinson's disease (PD) controls, significant reduced uptake was seen in the thalamus (9.0%), posterior frontal (8.9%), and inferior (9.9%), and anterior parietal (9.5%) cortex. A similar pattern of impaired uptake was seen in the clinically less/unaffected cerebral hemisphere in patients with CBD compared with normal controls, with a significant reduction in HMPAO uptake in the thalamus (6.8%), superior parietal (9.6%) and anterior parietal (7.6%), and posterior parietal (7.3%) cortex. This implies that the disease process is bilateral even in those cases with clinically unilateral disease. This widely available technique may be useful in the early diagnosis of CBD and in differentiation from other extrapyramidal disorders.
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PMID:Patterns of regional cerebral blood flow in corticobasal degeneration studied using HMPAO SPECT; comparison with Parkinson's disease and normal controls. 875 6

Corticobasal degeneration (CBD) is an adult-onset, progressive parkinsonian syndrome with strikingly asymmetrical features, and signs and symptoms referable to both cerebral cortex and basal ganglia. Although once considered rare, it is now recognized with increasing frequency during life. Eight patients with clinically diagnosed CBD and 8 age- and sex-matched patients with Parkinson's disease underwent high-field-strength magnetic resonance imaging (MRI) of the brain. MRIs were graded by a blinded neuroradiologist using a semiquantitative (0-3) scale. MRI of patients with CBD revealed significantly greater T2-weighted signal hypointensity in the putamena and globi pallidi, and ventricular enlargement. When specifically sought, asymmetrical cortical atrophy was identified in 5 of 8 CBD patients. Increased T2-weighted lenticular signal hypointensity, ventricular enlargement, and asymmetrical cortical atrophy are supportive MRI findings of CBD.
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PMID:Magnetic resonance imaging of corticobasal degeneration. 890 73

Corticobasal degeneration (CBD) is not rare disease, because in our clinic 13 patients were observed for the past 8 years, with ratio to those with Parkinson's disease being 1:18. Our clinical criteria of this disease consist of the combination of 1) limb-kinetic apraxia as cortical sign, 2) akinetic-rigid sign as extrapyramidal sign, 3) their marked asymmetry, and as additional findings, 4) the presence of grasp reflex, alien hand sign, reflex myoclonus, limb dystonia, and others, and 5) neuroimagings (MRI, SPECT) suggestive of asymmetric cortical lesions. There are reports indicating that clinical CBD was diagnosed as Pick's disease, progressive supranuclear palsy and Alzheimer's disease, pathologically. Therefore, more basic investigations, especially from molecular biology are necessary to discriminate these corticobasal complex disorders.
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PMID:[Cortico-basal degeneration]. 901 38

We used proton magnetic resonance spectroscopic imaging (1H-MRSI) to assess the in vivo cortical and subcortical neuronal involvement in progressive supranuclear palsy, Parkinson's disease and corticobasal degeneration. This technique permitted the simultaneous measurement of compounds containing N-acetylaspartate (NA), choline (Cho), creatine-phosphocreatine (Cre) and lactate, from four 15-mm slices divided into 0.84-ml single-volume elements. The study included 12 patients with progressive supranuclear palsy, 10 with Parkinson's disease, nine with corticobasal degeneration and 11 age-matched normal control subjects. Regions of interest were selected from the brainstem, caudate, thalamus, lentiform nucleus, centrum semiovale, and from frontal, parietal, precentral, temporal and occipital cortices. Progressive supranuclear palsy patients, compared with control subjects, had significantly reduced NA/Cre in the brainstem, centrum semiovale, frontal and precentral cortex, and significantly reduced NA/Cho in the lentiform nucleus. Corticobasal degeneration patients, compared with control subjects, had significantly reduced NA/Cre in the centrum semiovale, and significantly reduced NA/Cho in the lentiform nucleus and parietal cortex. There were no significant differences between Parkinson's disease patients and control subjects, or between patients groups in any individual region of interest. In the parietal cortex of corticobasal degeneration patients, NA/Cho was significantly reduced contralateral to the most affected side. There were statistically significant group differences in the regional pattern of NA/Cre and NA/Cho reduction, comparing normal control subjects with all patient groups, Parkinson's disease with corticobasal degeneration, and Parkinson's disease with progressive supranuclear palsy. Although the occurrence of significant groups differences does not imply that it is possible to differentiate between individual patients using 1H-MRSI in progressive supranuclear palsy and corticobasal degeneration, detection of specific cortical and subcortical patterns of neuronal involvement is possible with this technique. We suggest that this regional pattern of neuronal involvement found in progressive supranuclear palsy and corticobasal degeneration may help in the diagnostic evaluation of affected individuals.
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PMID:Proton magnetic resonance spectroscopic imaging in progressive supranuclear palsy, Parkinson's disease and corticobasal degeneration. 931 38

Corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP) are characterized by their unique clinical features and neuronal pathology. Although astrocytic plaques and tufts of abnormal fibers have been suggested to be specific histopathologic markers, recent studies have revealed significant clinicopathologic overlap between CBD and PSP. Based on the distinctive camera lucida profile of astrocytic inclusions on Gallyas-Braak silver staining, we found that astrocytic plaques and tufts of abnormal fibers did not coexist in the same patient among 30 cases of clinically diagnosed CBD, PSP and atypical Parkinson's disease. Using Tau immunohistochemistry it was difficult to verify the absence of tufts of abnormal fibers. A morphometric analysis revealed that the two groups classified by the presence or absence of astrocytic plaques and tufts of abnormal fibers exhibited significant differences in the density of ballooned neurons and neurofibrillary tangles and degeneration of the subcortical nuclei. Assessment using the NINDS neuropathologic criteria revealed that the cases with astrocytic plaques and tufts of abnormal fibers closely correspond to CBD and typical PSP, respectively. In addition, the cases lacking either of these two astrocytic inclusions had atypical PSP according to the NINDS criteria, and were associated with novel tau-positive astrocytes (spiny astrocytes). We thus conclude that astrocytic plaques and tufts of abnormal fibers are highly characteristic structures for CBD and typical PSP, respectively. We emphasize the importance of strict differentiation between different astrocytic inclusions not only for diagnosis, but also for further studies for elucidation of their role in the disease mechanisms of CBD and PSP.
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PMID:Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy. 979 5

Progressive supranuclear palsy (PSP) or Steele-Richardson-Olszewski syndrome is a neurodegenerative disease of middle and late age. It is under-diagnosed not only by general physicians but also by neurologists. The cause of PSP is not known. Exposure to toxins and viruses has been proposed in the aetiology of PSP without any concrete evidence. The features of PSP resemble those of Parkinson's disease and the two diseases are often confused. Corticobasal degeneration and multisystem atrophy are other differential diagnoses. Despite certain common features with Parkinson's disease, corticobasal degeneration, and mutisystem atrophy, there are important differences that help to differentiate it from these disorders.
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PMID:Progressive supranuclear palsy. 1082 45

Diagnostic accuracy has been addressed previously for Parkinson's disease in a brain bank collection, but accuracy of progressive supranuclear palsy (PSP) has not been addressed in a similar setting. Clinical and genetic features of pathologically confirmed cases of PSP were compared with misdiagnosed cases to determine ways to improve diagnostic accuracy. Medical records were reviewed for 180 cases sent to the Society of Progressive Supranuclear Palsy Brain Bank that had standardized neuropathologic evaluations as well as determination of apolipoprotein E and tau genotypes. Of the 180 cases studied, 137 had PSP and 43 had other pathologic diagnoses. Corticobasal degeneration (CBD), multiple system atrophy (MSA), and diffuse Lewy body disease (DLBD) accounted for 70% of the misdiagnosed cases. History of tremor, psychosis, dementia, and asymmetric findings were more frequent in misdiagnosed cases. The frequency of H1 tau haplotype (93 vs. 80%) and H1H1 genotype (86 vs. 66%) were significantly greater and APOE epsilon4 carrier state was significantly less (17 vs. 41 %) in PSP compared with misdiagnosed cases. Pathologic evaluation of clinically diagnosed PSP remains important for definitive diagnosis, and CBD, MSA, and DLBD are the disorders most likely to be misdiagnosed as PSP. Tremor, psychosis, early dementia, asymmetric findings, absence of H1 haplotype, and presence of APOE epsilon4 should raise questions about a diagnosis of PSP.
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PMID:Diagnostic accuracy of progressive supranuclear palsy in the Society for Progressive Supranuclear Palsy brain bank. 1450 69

Corticobasal degeneration was described in 1968 by Rebeiz, Kolodny and Richardson, who characterized the disease as a syndrome of asymmetric akinesis and rigidity, dystonia of the upper limb, apraxia, myoclonus and dementia. Atrophy of the frontal and parietal lobe, neuronal loss, gliosis and achromatic neurones (and nowadays astrocytic plaques) are the characteristic pathological features of the disease. Corticobasal degeneration is a rare or a rarely recognized disease and it is frequently misdiagnosed as Parkinson's disease. According to the Lang's criteria, corticobasal degeneration can be diagnosed in the presence of rigidity and one cortical symptom (apraxia, cortical sensory loss, alien hand) or in a patient with rigidity, dystonia and focal reflex myoclonus. Exclusion criteria are early dementia (as in primary degenerative dementias), early vertical gaze problems (as in progressive supranuclear palsy), resting tremor and good, sustained therapeutic response to levodopa (as in Parkinson's disease), severe autonomic problems (as in multiple system atrophy) and any pathology on imaging studies which might explain the clinical symptoms. It should be mentioned, that recently early dementia is recognized as an initial symptom of corticobasal degeneration. The authors present a case and review the literature to call attention to this disorder.
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PMID:[Clinical features of corticobasal degeneration]. 1588 98


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