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

We assessed the usefulness of routine MRI for the differential diagnosis of Parkinson's disease (PD) with "atypical" parkinsonian syndromes in everyday clinical practice. We studied routinely performed MRI in PD (n = 32), multiple system atrophy (MSA, n = 28), progressive supranuclear palsy (PSP, n = 30), and corticobasal degeneration (CBD, n = 26). From a preliminary analysis of 26 items, 4 independent investigators rated 11 easily recognizable MRI pointers organized as a simple scoring system. The frequency, severity and inter-rater agreement were determined. The total severity score was subdivided into "cortical", "putaminal", "midbrain", and "pontocerebellar" scores. The frequency of putaminal involvement (100%) and vermian cerebellar atrophy (45%) was significantly higher in MSA, but that of cortical atrophy (50%), midbrain atrophy and 3(rd) ventricle enlargement (75%) was higher in PSP and CBD. The median total score fairly differentiated "atypical" parkinsonian syndromes from PD (positive predictive value-PPV-90%). However, the median total score was unable to differentiate atypical parkinsonian syndromes each other. The "cortical" score distinguished CBD and PSP from MSA with a fair PPV (>90%). The PPV of the "putaminal" score was high (70%) for the differential diagnosis of MSA with PSP and CBD. The "midbrain" score was significantly higher in PSP and CBD compared to MSA. These results are in accordance with the underlying pathology found in these disorders and demonstrate that a simple MRI scoring procedure may help the neurologist to differentiate primary causes of parkinsonism in everyday practice.
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PMID:Routine MRI for the differential diagnosis of Parkinson's disease, MSA, PSP, and CBD. 1258 75

Several distinct clinical syndromes presenting with parkinsonism have been associated with subcortical neurofibrillary degeneration and the abnormal accumulation of hyperphosphorylated tau protein in the brain. Mutations of tau have been linked with a small number of autosomal dominantly inherited families who present with frontolimbic cognitive deficits, behavioural disorders, and Parkinson's syndrome. Some of the sporadic disorders (progressive supranuclear palsy [PSP] and corticobasal degeneration) have been referred to by molecular pathologists as primary tauopathies, implicating abnormalities of tau in their pathogenesis. We have identified a sporadic parkinsonian syndrome characterised by bradykinesia, a variable response to levodopa, and a mean duration of disease of 9 years, which resembles bodig (Parkinson's-dementia of Guam), and histologically has close similarities with both PSP and postencephalitic parkinsonism. Further characterisation of these cases frequently confused with Parkinson's disease may broaden the clinical spectrum of parkinsonian disorders linked with neurofibrillary tangle formation.
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PMID:Parkinson's syndrome associated with neurofibrillary degeneration and tau pathologic findings. 1450 53

We assessed the accuracy of clinical diagnosis of progressive supranuclear palsy (PSP, Steele-Richardson-Olszewski disease) and the validity of existing sets of clinical diagnostic criteria for PSP (see Appendix) using neuropathologically examined cases from the Queen Square Brain Bank for Neurological Disorders. Diagnosis of PSP was made by 40 different physicians, and 60 cases clinically diagnosed as PSP when last assessed in life were studied. In 47 cases (78%), the diagnosis of PSP was confirmed pathologically. False-positive diagnoses included Parkinson's disease with significant additional cortical Lewy body (n = 3) or Alzheimer (n = 1) pathology, multiple system atrophy (n = 4), and corticobasal degeneration, Pick's disease, motor neurone disease, cerebrovascular disease, and a sporadic case of frontotemporal dementia and parkinsonism linked to chromosome 17 (1 case each). Most cases of PSP were diagnosed accurately by neurologists at the final assessment. Although application of National Institute of Neurological Disorders and the Society for PSP possible category marginally improved the accuracy of initial clinical diagnosis, none of the existing operational criteria could significantly improve accuracy of the final clinical diagnosis.
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PMID:Accuracy of clinical diagnosis of progressive supranuclear palsy. 1497 73

We investigated the acquisition and long-term retention of new skills in patients with cortical (Alzheimer's disease, AD) and subcortical (progressive supranuclear palsy, PSP; Parkinson's disease, PD) degeneration. The motor skill task performance of the PD and PSP patients improved with training, but the improvement disappeared within a few months, whereas AD patients retained learned skills for 3-18 months. The results of our experiments show that subcortical dysfunction induces a retention deficit for newly learned motor skills. Our present study suggests that a normal striatum is necessary for the formation of long-lasting motor skills, and that the striatum plays an important role as a motor skill consolidation system.
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PMID:Deficits in long-term retention of learned motor skills in patients with cortical or subcortical degeneration. 1535 34

72 consecutive patients with suspected parkinsonian syndromes (PS) were studied by dopamine transporter (DAT) and D2 receptor SPECT in order to evaluate the accuracy of combined SPECT imaging. In the follow-up, the patients were diagnosed as having Parkinson's disease (PD, n = 25), dementia with Lewy bodies (DLB, n = 6), multiple system atrophy (MSA, n = 13), progressive supranuclear palsy (PSP, n = 8), corticobasal degeneration (CBD, n = 9), and essential tremor (ET, n = 11). Using the iteratively estimated optimal cutoffs, DAT was reduced in 57/61 PS patients, whereas all ET patients were identified as "normal". Reduced D2 receptor binding had 7/13 patients with MSA, 6/8 patients with PSP, 2/9 patients with CBD and no ET, PD or DLB patients. FP-CIT SPECT allows an accurate detection of nigrostriatal affection in neurodegenerative PS. IBZM SPECT is useful to approve the diagnosis of PSP and MSA although a normal finding cannot exclude an atypical PS. IBZM SPECT seems to be of restricted value in CBD.
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PMID:Combined 123I-FP-CIT and 123I-IBZM SPECT for the diagnosis of parkinsonian syndromes: study on 72 patients. 1537 77

Deposition of amyloid-beta, the fibrillogenic product of the cell surface protein AbetaPP (amyloid-beta protein precursor), occurs in the cerebral cortex of patients with Dementia with Lewy bodies (DLB). Amyloid deposition, basically in the form of senile plaques, occurs not only in the common form (DLBc), which is defined by changes consistent with diffuse Lewy body disease accompanied by Alzheimer's disease (AD), but also in the pure form (DLBp), in which neurofibrillary tangles are absent. The present study analyses the expression of AbetaPP mRNA isoforms with (AbetaPP751 and AbetaPP770) and without (AbetaPP695) the Kunitz-type serine protease inhibitor (KPI) domain, in the cerebral cortex in DLBc (n=4), DLBp (n=4), Parkinson's disease (PD, n=5), AD (n=3 stages I-IIA, and n=4 stage VC of Braak and Braak), amyloid angiopathy (AA, n=2) and progressive supranuclear palsy (PSP, n=4) compared with age-matched controls (n=6). For this purpose, TaqMan RT-PCR assay was used on frozen post-mortem samples of the frontal cortex (area 8) obtained with short post-mortem delays (8.29+/-4.57 h) and strict RNA preservation (A260/280 of 1.78+/-0.15). A 3.66-fold, 6.67-fold, 4.28-fold and 5.24-fold increases, in the (AbetaPP751+AbetaPP770)/AbetaPP695 mRNA ratio were found in DLBc, DLBp, AD stage VC and AA, respectively, when compared with controls. No modifications in the ratio were found in PD, AD stage I-IIA and PSP. These findings suggest that alternative splicing of the AbetaPP mRNA may play a role in betaA4 amyloidogenesis in DLBp, DLBc, AD stage VC and Amyloid angiopathy.
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PMID:Amyloid-beta deposition in the cerebral cortex in Dementia with Lewy bodies is accompanied by a relative increase in AbetaPP mRNA isoforms containing the Kunitz protease inhibitor. 1567 Jun 42

Decreased cardiac uptake of meta-iodobenzylguanidine (MIBG) on [123I]MIBG myocardial scintigraphy has been reported in Parkinson's disease (PD) and dementia with Lewy bodies (DLB). We hypothesized that cardiac sympathetic denervation might account for the pathomechanism. To elucidate the extent, frequency and pattern of cardiac sympathetic nerve involvement in Lewy body disease and related neurodegenerative disorders, we immunohistochemically examined heart tissues from patients with PD (n=11), DLB (n=7), DLB with Alzheimer's disease (DLB/AD; n=4), multiple system atrophy (MSA; n=8), progressive supranuclear palsy (PSP; n=5), pure AD (n=10) and control subjects (n=5) together with sympathetic ganglia from patients with PD (n=5) and control subjects (n=4), using an antibody against tyrosine hydroxylase (TH). TH-immunoreactive nerve fibers in the hearts had almost entirely disappeared in nearly all the patients with PD, DLB and DLB/AD, whereas they were well preserved in all the patients with PSP and pure AD as well as in all except for one patient with MSA. In PD, neurons in the sympathetic ganglia were preserved in all except for one patient. Decreased cardiac uptake of MIBG in Lewy body disease reflects actual cardiac sympathetic denervation, which precedes the neuronal loss in the sympathetic ganglia.
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PMID:Cardiac sympathetic denervation precedes neuronal loss in the sympathetic ganglia in Lewy body disease. 1593 69

We aimed to evaluate the potential of the cerebrospinal fluid (CSF) axonal damage biomarker NfH(SMI35) in the laboratory-supported differential diagnosis of parkinsonian syndromes. Patients with idiopathic Parkinson's disease (PD; n = 22), multiple-system atrophy (MSA; n = 21), progressive supranuclear palsy (PSP; n = 21), corticobasal degeneration (CBD; n = 6), and age-matched controls (n = 45) were included. CSF levels of NfH(SMI35) were measured using ELISA. Levels of CSF NfH(SMI35) were elevated in PSP compared to PD and controls (P < 0.05 each). They were also significantly higher in MSA than in PD and controls (P < 0.05 each). NfH(SMI35) differentiated PD from PSP with a sensitivity of 76.5% and a specificity of 94.4%. Axonal damage as measured by CSF NfH(SMI35) is most prominent in the more rapidly progressive syndromes PSP and MSA as compared to PD or CBD. CSF NfH(SMI35) may therefore be of some value for the laboratory-supported differential diagnosis of atypical parkinsonian syndromes.
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PMID:Neurofilament heavy-chain NfH(SMI35) in cerebrospinal fluid supports the differential diagnosis of Parkinsonian syndromes. 1701 9

Pathological changes of the superior cerebellar peduncle (SCP) can occur in PSP. We assessed the clinical history and signal changes in the SCP on fluid-attenuated inversion recovery (FLAIR) images of 12 patients with clinically probable PSP. Three control groups were studied: Parkinson's disease (PD), multiple system atrophy with predominant parkinsonian features (MSA-P), and healthy controls. Three patients who had clinically probable PSP showed increased FLAIR signals within the SCP. No subject with PD or MSA-P showed any signal changes of the SCP. The signal changes in the SCP on FLAIR may be one indicator for differentiating PSP from other parkinsonian diseases.
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PMID:Signal changes of superior cerebellar peduncle on fluid-attenuated inversion recovery in progressive supranuclear palsy. 1748 36

A case is described of a patient with levodopa responsive parkinsonism, dyskinesia, and off periods who underwent bilateral deep brain stimulation of the subthalamic nuclei (DBS-STN) with good result. As the disease progressed, the patient fit diagnostic criteria for progressive supranuclear palsy parkinsonism (PSP-P). Benefit of DBS for the parkinsonian phenomena is still apparent 4 years later. That DBS-STN provided targeted symptom relief in this unusual patient suggests that the parkinsonism of PSP-P and that of Parkinson's disease (PD) may share a common pathophysiologic mechanism. The presence of dystonic features in this illness raises the possibility that the globus pallidus might also have been an effective target.
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PMID:Subthalamic stimulation improves levodopa responsive symptoms in a case of progressive supranuclear palsy. 1782 99


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