Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0030567 (Parkinson's disease)
 63,064 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

125I-Nerve growth factor (NGF) binding sites were analyzed by autoradiography in the striatum of 3 control subjects, 3 patients with Parkinson's disease and 3 patients with progressive supranuclear palsy. A high level of 125I-NGF binding was observed (0.3-0.4 fmol/mg of tissue equivalent) in the striatum and the nucleus basalis of Meynert of control patients. Pockets of lower 125I-NGF binding corresponding to acetylcholinesterase-poor striosomes were detected in the striatum of control subjects and patients with Parkinson's disease or progressive supranuclear palsy. When compared to controls, the density of 125I-NGF binding sites was reduced by 30% in the striatum of patients with progressive supranuclear palsy but not reduced in that of patients with Parkinson's disease. 125I-NGF binding was not significantly decreased in the nucleus basalis of Meynert in either diseases. Since NGF receptors are thought to be localized on cholinergic neurons in the striatum, the decrease in NGF binding is compatible with the loss of cholinergic neurons reported in the striatum from PSP patients.
 ...
PMID:Loss of striatal high affinity NGF binding sites in progressive supranuclear palsy but not in Parkinson's disease. 789 88

The Steel-Richardson-Olszewski syndrome (progressive supranuclear palsy: PSP) was described over a quarter of a century age. Although the full expressed form is very typical, it is overlooked due to unusual ways without axial dystonia and opthalmic signs, with akinesia and dysequilibrium. The many reports of PSP suggested that the abnormalities of it were vaster than Parkinson's disease. The abnormalities of neurotransmitters or neuromodulators were found not only dopamine system but also serotonin and acetylcholine system. On the basis of them, the various trials of neurotransmitter replacement were done without very successful results so far. Transplantation and nerve growth factor are also tried to treat PSP now.
 ...
PMID:[Steele-Richardson-Olszewski syndrome]. 790 88

PSP is associated with a widespread cholinergic deficit likely corresponding to a loss in cholinergic neurons. The cholinergic damage dramatically affects the basal ganglia and specific cell groups of the mesencephalon and pons. This provides an anatomically defined basis for motor and supranuclear oculomotor syndromes characteristic of PSP. Unlike Alzheimer's disease and Parkinson's disease with dementia, the disease is not associated with a marked cholinergic deficiency in the cerebral cortex. Various peptides are present at normal concentrations in extrapyramidal and limbic subcortical areas in brains of patients with PSP. Of particular interest, is somatostatin, the levels of which are subnormal in cerebral cortex of patients with dementia of Alzheimer' or Parkinson's disease type.
 ...
PMID:Cholinergic and peptidergic systems in PSP. 796 88

The age-adjusted prevalence of PSP as measured in central New Jersey is 1.5 cases per million population, about 1% of that of Parkinson's disease. It incidence is 3-4 new cases per million population per year, similar to that of such better-known illnesses as myasthenia gravis, the hereditary ataxias as a group and Tourette syndrome. Median actuarially adjusted survival after symptom onset is 5.9-6.9 years. PSP appears to favor no geographical, racial, ethnic or occupational group, though there is anecdotal evidence for hydrocarbon exposure as a candidate etiologic factor. No familial cases of typical PSP have been proven. The one formal case-control study failed to implicate any particular causal agent and the rural predilection of PD appears to be absent in PSP. Better diagnostic methods, more multi-center organization, additional case-control studies and new etiologic hypotheses are needed in the epidemiological investigation of PSP.
 ...
PMID:The epidemiology of PSP. 796 91

OBJECTIVE--To analyse the natural history of progressive supranuclear palsy (PSP or Steele-Richardson-Olszewski syndrome) and clinical predictors of survival in 24 patients with PSP confirmed by necropsy, who fulfilled the NINDS criteria for a neuropathological diagnosis of typical PSP. METHODS--Patients were selected from the research and clinical files of seven medical centres involving tertiary centres of Austria, England, France, and the United States. Clinical features were analysed in detail. The patients' mean age at onset of PSP was 63 (range 45-73) years. RESULTS--The most frequent clinical features (occurring in at least 75% of the patients) were early postural instability and falls, vertical supranuclear palsy, akinetic-rigid predominant parkinsonian disorder characterised by symmetric bradykinesia and axial rigidity unrelieved by levodopa, pseudobulbar palsy, and frontal release signs. Occasionally, segmental dystonia or myoclonus were described, but neither aphasia nor alien limb syndrome was reported. Fractures occurred in 25% of the patients but were unrelated to the severity of the gait or to the presence of falls. Median survival time was 5.6 (range 2-16.6) years. Onset of falls during the first year, early dysphagia, and incontinence predicted a shorter survival time. Age at onset, sex, early onset of dementia, vertical supranuclear palsy, or axial rigidity had no effect on prognosis of survival. Pneumonia was the most common immediate cause of death. PSP was most often clinically misdiagnosed as Parkinson's disease. Errors in diagnosis suggest that PSP is underdiagnosed. CONCLUSION--Progressive onset of early postural instability with falls or supranuclear vertical palsy in the fifth decade, should suggest the diagnosis of PSP. Onset of falls during the first year are emphasised, as they could lead to an early diagnosis and influence the prognosis of patients with PSP. Whether appropriate treatment of the dysphagia could prolong the survival of PSP patients needs to be explored.
 ...
PMID:Natural history of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) and clinical predictors of survival: a clinicopathological study. 864 26

Ceruloplasmin (CP), the major plasma anti-oxidant and copper transport protein, is synthesized in several tissues, including the brain. We compared regional brain concentrations of CP and copper between subjects with Alzheimer's disease (AD, n = 12), Parkinson's disease (PD, n = 14), Huntington's disease (HD, n = 11), progressive supranuclear palsy (PSP, n = 11), young adult normal controls (YC, n = 6) and elderly normal controls (EC, n = 7). Mean CP concentrations were significantly increased vs. EC (P < 0.05) in AD hippocampus, entorhinal cortex, frontal cortex, and putamen. PD hippocampus, frontal, temporal, and parietal cortices, and HD hippocampus, parietal cortex, and substantia nigra. Immunocytochemical staining for CP in AD hippocampus revealed marked staining within neurons, astrocytes, and neuritic plaques. Increased CP concentrations in brain in these disorders may indicate a localized acute phase-type response and/or a compensatory increase to oxidative stress.
 ...
PMID:Increased regional brain concentrations of ceruloplasmin in neurodegenerative disorders. 895 22

The accuracy of the clinical diagnosis of corticobasal degeneration (CBD) is unknown. To determine its diagnostic accuracy, we presented 105 cases with known neuropathologic diagnoses, including CBD (n = 10), progressive supranuclear palsy (PSP, n = 24), Parkinson's disease (n = 15), diffuse Lewy body disease (n = 14), multiple system atrophy (n = 16), postencephalitic parkinsonism (n = 7), Pick's disease (n = 7), Creutzfeldt-Jakob disease (n = 4), Alzheimer's disease (n = 4), vascular parkinsonism (n = 3), and Whipple's disease (n = 1), as clinical vignettes to six neurologists unaware of the autopsy findings. Reliability was measured with the kappa statistics. The neurologists' clinical diagnoses were compared with clinicopathologic diagnoses for sensitivity, specificity, and positive predictive values at first and last clinic visits. The group reliability for the diagnosis of CBD significantly improved from moderate for the first visit (mean = 34 months after onset) to substantial for the last (68 months after onset). For the first visit, mean sensitivity for CBD was low (35%), but specificity was near-perfect (99.6%). For the last visit, mean sensitivity minimally increased (48.3%), and specificity remained stable. False-negative misdiagnoses mainly occurred with PSP. False-positive diagnoses were rare. The extremely low sensitivity of the clinical diagnosis of CBD suggests that this disorder is markedly underdiagnosed. Although the validity of the clinical diagnosis might have been improved if neurologists could have examined these patients, more important is that this disorder was misdiagnosed by the primary neurologists. In our data set, the best predictors for the diagnosis of CBD included limb dystonia, ideomotor apraxia, myoclonus, and asymmetric akinetic-rigid syndrome with late onset of gait or balance disturbances.
 ...
PMID:Accuracy of the clinical diagnosis of corticobasal degeneration: a clinicopathologic study. 900 6

Positron emission topographic studies on local cerebral glucose metabolism in Parkinson's disease (PD) including our own data were reviewed. In our 18F-FDG PET studies, local or global metabolic change was not found in 9 patients with non-demented PD, with respect to 5 normal controls. Moreover, there was not an apparent difference between severe PD group (Hoehn-Yahr III-IV) and mild PD group (Hoehn-Yahr I-II). In other PD patients with dementia or autonomic failure, parietal dominant hypometabolism was found likely to those of Alzheimer disease, but lenticular nucleus was well preserved. Furthermore 18F-FDG PET findings of atypical parkinsonian syndromes, such as SND and PSP were reviewed. They showed relative hypometabolism in the basal ganglia in PET images. PET study with FDG provides a clue to differential diagnosis of parkinsonian patients.
 ...
PMID:[18F-fluorodeoxyglucose positron emission tomography in Parkinson's disease]. 901 54

The difficulty in differentiating progressive supranuclear palsy (PSP, also called Steele-Richardson-Olszewski syndrome) from other related disorders was the incentive for a study to determine the clinical features that best distinguish PSP. Logistic regression and classification and regression tree analysis (CART) were used to analyse data obtained at the first visit from a sample of 83 patients with a clinical history of parkinsonism or dementia confirmed neuropathologically, including PSP (n = 24), corticobasal degeneration (n = 11), Parkinson's disease (PD, n = 11), diffuse Lewy body disease (n = 14). Pick's disease (n = 8) and multiple system atrophy (MSA, n = 15). Supranuclear vertical gaze palsy, moderate or severe postural instability and falls during the first year after onset of symptoms classified the sample with 9% error using logistic regression analysis. The CART identified similar features and was also helpful in identifying particular attributes that separate PSP from each of the other disorders. Unstable gait, absence of tremor-dominant disease and absence of a response to levodopa differentiated PSP from PD. Supranuclear vertical gaze palsy, gait instability and the absence of delusions distinguished PSP from diffuse Lewy body disease. Supranuclear vertical gaze palsy and increased age at symptom-onset distinguished PSP from MSA. Gait abnormality, severe upward gaze palsy, bilateral bradykinesia and absence of alien limb syndorme separated PSP from corticobasal degeneration. Postural instability successfully classified PSP from Pick's disease. The present study may help to minimize the difficulties neurologists experience when attempting to classify these disorders at early stages.
 ...
PMID:Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders? A clinicopathological study. 905 98

Positron emission tomography (PET) with [18F] fluorodopa (FD) assesses nigrostriatal function in vivo. Measurements deriving from FD PET have direct lineal relationship to the nigral cell count in normal ageing and in Parkinson's disease (PD), although in PD the progression of the decline in nigrostriatal dopaminergic function is faster than the controls. The functional anatomy of the movements has been studied using PET and SPECT. The supplementary motor area (SMA) dysfunction is responsible for bradykinesia in Parkinson. The SMA is undereactive in PD during self-generated movements, and this underactivation is reversed by the administration of apomorphine and modulate by the stereotaxic ventral pallidotomy. PET has been used, also, to attempt the differentiation of PD, multiple system atrophy (MSA) and PSP, in vivo, using a number of different radioligands (opiod, dopamine receptor binding). [11C] raclopride PET and [123I] IBF SPECT seemed a promising agents to assessing D2 receptor status in humans. Studies of D2 status have demonstrated normal or increased D2 receptor density in PD and decreased receptor density in PSP and MSA. Using SPECT has been demonstrated marked differences of the DA transporters located on dopaminergic terminals in the striatum in healthy controls and PD patients. Also the correlation of SPECT measures of transporters of DA and motor severity suggests that this may be a useful marker of disease severity in PD.
 ...
PMID:[PET and SPECT in Parkinson's disease]. 928 Jun 81


<< Previous 1 2 3 4 5 6 7 Next >>