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At least 1,549 records · Page 86Linked to original sources
PubMed2004

A functional polymorphism regulating dopamine beta-hydroxylase influences against Parkinson's disease.

A functional --1021C --> T polymorphism in the dopamine beta-hydroxylase gene has been demonstrated to regulate plasma DBH activity. We report that individuals with genetically determined low serum DBH activity (genotype T/T) have protection against Parkinson's disease (p = 0.01). In particular, we observed an underrepresentation of the T/T genotype odds ratio = 0.46 (CI = 0.27-0.8). Rather than identifying a haplotype, or a marker in linkage disequilibrium with the risk variant, this to our knowledge is the first report directly linking PD susceptibility with a proven functional variant.

Animals↗
PubMed2003

Secondary progressive chronic manganism associated with markedly decreased striatal D2 receptor density.

We describe a patient with chronic manganism due to intoxication 40 years ago. Whereas previous reports on acute or subacute intoxication have shown no or only small reductions in striatal D2 receptor density, we found markedly decreased D2 receptor density using (18)F-methylspiperone PET in this very late stage of chronic manganism, supporting the hypothesis that manganese intoxication may trigger a neurodegenerative disease process.

Aged↗
PubMed2003

Diagnostic potential of acoustic startle reflex, acoustic blink reflex, and electro-oculography in progressive supranuclear palsy: a prospective study.

We carried out a prospective study to analyze the diagnostic potential of acoustic startle reflex (ASR), acoustic blink reflex (ABR) and electro-oculography (EOG) in early stages of atypical parkinsonian syndrome. The study was carried out in a consecutive series of 41 patients clinically diagnosed as atypical parkinsonism (mean time from first symptoms of 38 months and follow-up of 26 months). The three procedures were carried out immediately after the first clinical evaluation. ASR and ABR were elicited by auditory stimuli while the patient was attending to a simple reaction time task. Outcome measures were: ASR (absence/presence, latency), ABR (absence/presence, latency) and EOG (suggestive/not suggestive of progressive supranuclear palsy [PSP]). Final clinical diagnosis was carried out by two neurologists blind to the neurophysiological results. A study of diagnostic sensitivity and odds ratio (OR) calculation for the PSP diagnosis was carried out. Neurophysiological examination showed the following sensitivity/specificity (%) for the diagnosis of PSP: ASR: 100/89; ABR 85/89; EOG 100/72. OR values were: ASR: 0.011; ABR: 0.037; EOG: 0.038. The three tests taken simultaneously showed a sensitivity of 100% and a specificity of 95%. The three neurophysiological tests investigated provided sensitive and specific measures with predictor value in early stages of atypical parkinsonian syndrome.

Aged↗
PubMed2001

Focal myopathy as a cause of anterocollis in Parkinsonism.

We present a patient with levodopa-responsive Parkinsonism who developed disproportionate anterocollis over a period of 4 to 5 weeks. A neurological evaluation, including EMG and muscle biopsy, demonstrated the presence of a focal myopathy typical of the so-called "dropped head syndrome" or "isolated neck extensor myopathy." Parkinsonian patients presenting with disproportionate anterocollis should be evaluated for focal myopathy, as this may represent one possible cause.

Aged↗
PubMed2007

Regional metabolic changes in parkinsonian patients with normal dopaminergic imaging.

Dopaminergic imaging has been found to be normal in approximately 15% of parkinsonian patients enrolled in neuroprotective trials. We used (18)F-fluorodeoxyglucose positron emission tomography (FDG PET) to determine the metabolic basis for this finding. We reviewed scans from 185 patients with clinical signs of Parkinson's disease (PD) who underwent (18)F-fluorodopa PET imaging for diagnostic confirmation. Of this group, 27 patients (14.6%) had quantitatively normal scans; 8 of these patients were additionally scanned with FDG PET. Pattern analysis was performed on an individual scan basis to determine whether the metabolic changes were consistent with classic PD. Computer-assisted single-case assessments of the FDG PET scans of these 8 patients did not disclose patterns of regional metabolic change compatible with classical PD or an atypical parkinsonian variant. Similarly, network quantification revealed that PD-related pattern expression was not elevated in these patients as it was in an age- and duration-matched cohort with classical PD (P < 0.0001). None of these patients developed clinical signs of classical PD or of an atypical parkinsonian syndrome at a follow-up visit conducted 3 years after imaging. The results suggest that parkinsonian subjects with normal dopaminergic imaging do not have evidence of classical PD or an atypical parkinsonian syndrome.

Brain↗
PubMed1998

Differential diagnosis of parkinsonism with [18F]fluorodeoxyglucose and PET.

The clinical differentiation between typical idiopathic Parkinson's disease (IPD) and atypical parkinsonian disorders (APD) is complicated by the presence of signs and symptoms common to both forms of parkinsonism. Metabolic brain imaging with [18F]fluorodeoxyglucose (FDG) and positron emission tomography (PET) may be a useful adjunct in differentiating APD from IPD. To explore this possibility, we studied 48 parkinsonian patients suspected as having possible APD because of a deteriorating response to dopaminergic treatment, the development of autonomic dysfunction, or both. A group of 56 patients with likely IPD served as control subjects. We used quantitative FDG/PET to measure regional rates of cerebral glucose use in IPD and APD patients. We used discriminant analysis to categorize IPD and APD patients based on their regional metabolic data. We found that a linear combination of caudate, lentiform, and thalamic values accurately discriminated APD from IPD patients (p < 0.0001). Significant metabolic abnormalities were present in the striatum and the thalamus of 36 of 48 (75%) APD patients. Our findings show that measurements of regional glucose metabolism can be used to discriminate patients with suspected APD from their counterparts with classic IPD. FDG/PET may be a useful adjunct to the clinical examination in the differential diagnosis of parkinsonism.

Aged↗
PubMed1998

Asymmetry of basal ganglia glucose metabolism and dopa responsiveness in parkinsonism.

We investigated, by positron emission tomography (PET) with [18F]fluoro-2-deoxy-d-glucose (FDG) (FDG-PET), brain glucose metabolism in 19 patients with parkinsonian features. We compared local pattern of FDG uptake and asymmetry indexes in patients with therapeutic response to levodopa (L-dopa) (group 1, presumed Parkinson's disease, n = 9) and patients without L-dopa therapeutic response (group 2, presumed striatonigral degeneration, n = 10). Limb dystonia was present in 11% of patients in group 1 and in 40% of patients in group 2. Asymmetry in basal ganglia metabolism was distributed differently in the two groups (analysis of variance, p < 0.04). In superior and inferior putamen, superior and middle caudate, ventral striatum, and inferior thalamus, relative reduction in metabolism on the side contralateral to predominant parkinsonian signs was associated with L-dopa unresponsiveness. On the contrary, in middle caudate, ventral striatum, and inferior thalamus, a relative increase in metabolism on the side contralateral to the predominant side, parkinsonian signs were found in L-dopa-responsive patients. Our FDG-PET study using simple statistical procedures demonstrates inverse asymmetry of basal ganglia glucose metabolism in parkinsonian patients grouped on the sole basis of L-dopa responsiveness.

Adult↗
PubMed2001

Autoregulatory cerebral vasodilation occurs during orthostatic hypotension in patients with primary autonomic failure.

It is unclear whether patients with autonomic failure autoregulate cerebral blood flow during hypotension. The objective in this study was to examine cerebral autoregulatory capacity in patients with autonomic failure by studying changes in middle cerebral artery blood flow velocity using transcranial Doppler ultrasonography before, during, and after tilt-induced hypotension. Nine patients with primary autonomic failure were evaluated. Mean arterial pressure and middle cerebral artery blood flow velocity were simultaneously recorded while the patients were in the supine position, during 60 degrees head-up tilt, and after they were returned to the horizontal position. The results were as follows: during tilt-induced hypotension, mean arterial pressure decreased significantly more than middle cerebral artery mean blood flow velocity (58% versus 36%, p <0.0002). After return to the horizontal position, mean arterial pressure returned to baseline, and middle cerebral artery blood flow velocity transiently increased above pretilt value (p <0.02). It is concluded that cerebral autoregulatory vasodilation occurs in patients with autonomic failure. This was demonstrated by a more pronounced decline in mean arterial pressure than in middle cerebral artery blood flow velocity during hypotension and by a transient increase in middle cerebral artery blood flow velocity (ie, hyperemic response) after blood pressure was restored.

Aged↗
PubMed1999

Patients' choice of portable folding chairs to reduce symptoms of orthostatic hypotension.

Patients with neurogenic orthostatic hypotension may use portable folding chairs to prevent or reduce symptoms of low blood pressure. However, a concomitant movement disorder may limit the use of these chairs in daily living. In this prospective study, 13 patients with orthostatic hypotension, balance disturbance associated with motor disability, or both examined three commercially available portable folding chairs. A questionnaire was used to document the characteristics in chair design that were relevant for satisfactory use to these patients. Armrests, seat width, and an adjustable sitting height were found to be important features of a portable folding chair. One chair was selected by 11 of 13 patients to fit most needs.

Aged↗
PubMed2000

Alpha-synuclein and Parkinson's disease.

The involvement of alpha-synuclein in neurodegenerative diseases was first suspected after the isolation of an alpha-synuclein fragment (NAC) from amyloid plaques in Alzheimer's disease (AD). Later, two different alpha-synuclein mutations were shown to be associated with autosomal-dominant Parkinson's disease (PD), but only in a small number of families. However, the discovery that alpha-synuclein is a major component of Lewy bodies and Lewy neurites, the pathological hallmarks of PD, confirmed its role in PD pathogenesis. Pathological aggregation of the protein might be responsible for neurodegeneration. In addition, soluble oligomers of alpha-synuclein might be even more toxic than the insoluble fibrils found in Lewy bodies. Multiple factors have been shown to accelerate alpha-synuclein aggregation in vitro. Therapeutic strategies aimed to prevent this aggregation are therefore envisaged. Although little has been learned about its normal function, alpha-synuclein appears to interact with a variety of proteins and membrane phospholipids, and may therefore participate in a number of signaling pathways. In particular, it may play a role in regulating cell differentiation, synaptic plasticity, cell survival, and dopaminergic neurotransmission. Thus, pathological mechanisms based on disrupted normal function are also possible.

14-3-3 Proteins↗
PubMed1998

Expression of low-affinity neurotrophin receptor p75NTR in the peripheral nervous system of human neuropathies.

Expression of low-affinity neurotrophin receptor (p75NTR) was immunohistochemically examined in the peripheral nerve trunks, dorsal root ganglia, sympathetic nerve ganglia and spinal cords in various human neurological diseases manifesting peripheral neuropathies. p75NTR was expressed in the nerves with axonal degeneration, and was also prominent in the nerves with newly regenerating axons. In contrast, axonal pathology tended to reduce the expression of p75NTR in the neuronal perikarya of the dorsal root ganglion and sympathetic nerve ganglion neurons. In the ventral and lateral horn cells, the p75NTR immunoreactivity was not detected in the normal and diseased nerves except for amyloid polyneuropathy. These p75NTR expressions in the diseased human peripheral nervous tissues would be regulated by an underlying pathology-related process, and could play a role in peripheral nerve repair.

Aged↗