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)

The most prominent symptom of Shy-Drager syndrome is the asympathicotonic orthostatic (postural) hypotension, which is associated with a number of additional autonomic and neurological disturbances: disorders of micturition, sphincter disturbances, impotence, anhidrosis, hypokinesia, rigidity, pyramidal symptoms, cerebellar dysfunction and nuclear pareses due to anterior horn cell degeneration. The various disorders are not caused by ischemia or hypotension, but they represent parts of a multisystemic disease of still unknown etiology. According to different extension and neuropathological criteria it has been suggested to distinguish two types of neurogenic (idiopathic) orthostatic hypotension. Moreover, differential diagnosis of the Shy-Drager syndrome has to consider postural hypotension occuring as a symptom in some neuropathies and Parkinson's disease. Symptomatology, course, prognosis and treatment of Shy-Drager syndrome are described, as well as relevant findings of apparative investigations, pharmacological and hemodynamic tests and neuropathological findings in autopsied cases reported in the literature. This review was initiated by two clinically investigated cases of Shy-Drager syndrome.
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PMID:[The Shy-Drager syndrome (author's transl)]. 24 13

The changes found in the brain and spinal cord and vegetative ganglias in one observation of nerogenic orthostatic hypotension or Shy-Drager syndrome are described. The morphological picture corresponds to a variant of Shy-Drager syndrome with a wide involvement of the nervous system at its various levels. Its is concluded that a thorough examination of the nervous system (including the spinal cord and vegetative ganglia) should be done in all cases of Shy-Drager syndrome and clinically similar diseases accompanied by vegetative disorders, for instance in shaking palsy.
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PMID:[Morphology of the Shy-Drager syndrome]. 49 88

[3H]Spiperone binding was investigated in the caudate nucleus, substantia nigra (s. nigra) and frontal cortex of control subjects and of patients with Parkinson's disease and the Shy-Drager syndrome. Binding sites for [3H]spiperone were interpreted as dopamine receptors in caudate and s. nigra, and as 5-hydroxytryptamine (5-HT) receptors in frontal cortex. Scatchard analysis showed that the Bmax (maximal number of binding sites) in caudate was similar in the 3 groups, whereas in s. nigra the Bmax was reduced by approximately 60% in both Parkinsons disease and Shy-Drager syndrome. The dissociation constant (Kd) for [3H]spiperone binding in s. nigra was similar in the 3 groups. In caudate nucleus, the Kd was similar in control and Parkinson groups; however, there was a significant increase in the dissociation constant in the caudate nucleus from cases of Shy-Drager syndrome. No differences in binding characteristics were observed in the frontal cortex. These results are taken to reflect a loss of dopamine receptor sites in the s. nigra in both Parkinson's disease and Shy-Drager syndrome, and a reduced affinity of dopamine receptor sites in the caudate nucleus in Shy-Drager syndrome.
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PMID:Alterations in [3H]spiperone binding in human caudate nucleus, substantia nigra and frontal cortex in the Shy-Drager syndrome and Parkinson's disease. 52 36

Studies of pulse rate and blood pressure responses to graded intravenous infusions of noradrenaline and dopamine were performed on five patients with Parkinson's disease, five with the Shy-Drager syndrome and seven healthy subject. Cardiovascular reflex responses to standing and to the Valsalva manoeuvre were found to be preserved in all patients with Parkinson's disease but to be grossly defective or absent in all with the Shy-Drager syndrome. Each subject received separate intravenous infusions of L-noradrenaline and dopamine, delivered at increasing rates, until a 30% rise in systolic blood pressure was achieved. Heart rate decreased during pressor responses to noradrenaline in control subjects and patients with Parkinson's disease, but increased in those with the Shy-Drager syndrome. Heart rate increased during pressor responses to dopamine in all subjects. Compared to control subjects supersensitivity to noradrenaline was observed both in patients with Parkinson's disease and, to a greater extent, in those with the Shy-Drager syndrome. Subsensitivity to dopamine was observed in patients with Parkinson's disease, but supersensitivity in those with the Shy-Drager syndrome.
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PMID:Blood pressure responses to noradrenaline and dopamine infusions in Parkinson's disease and the Shy-Drager syndrome. 78 43

Postural changes in blood pressure were recorded in all 391 patients suffering from Parkinson's syndrome over a period of six years. Intraarterial blood pressure studies were carried out in those with significant postural hypotension. Histological examination of the entire central nervous system and the sympathetic ganglia was performed in six patients suffering from idiopathic Parksinson's disease. Five of the six patients had Lewy bodies in the sympathetic ganglia. Loss of nerve cells was noted in the sympathetic ganglia in those patients that demonstrated postural hypotension. The severity of the lesions in the ganglia correlated with the severity of postural hypotension in idiopathic Parkinson's disease, One case of Shy-Drager syndrome was similarly studied to demonstrate the differences in spinal cord and sympathetic ganglia lesions in the two conditions.
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PMID:Postural hypotension in idiopathic Parkinson's disease. Etiopathology. 101 28

We report an autopsied case of Parkinson's disease manifesting Shy-Drager syndrome. At the age of 63 years, the patient noticed an onset of progressive orthostatic dizziness, which was followed by constipation, dysuria, and sexual impotence. When he was 66 years old, syncopal attack for a few minutes, tremor in the bilateral hands, and memory disturbance developed. On admission, his blood pressure was 142/72 mmHg in supine position, which fell to 58/42 mmHg on standing with appropriate increase of heart rate. Neurological examination revealed hallucination, memory disturbance, masked face, muscular rigidity, bradykinesia, mild postural tremor, and autonomic dysfunction including severe orthostatic hypotension, hypohydrosis, constipation, dysuria, and sexual impotence. Electroencephalogram showed diffuse slowing. Brain CT demonstrated absence of severe atrophy of the cerebellum, and brain stem. Pharmacological study revealed denervation hypersensitivity to the intravenously administrated noradrenaline. A diagnosis of Shy-Drager syndrome was made, and he was treated with anti parkinsonian drugs. However, no improvement was observed in his clinical symptoms. Seven months later, he died of pneumonia. Neuropathological examination revealed marked neuronal cell loss and gliosis in the substantia nigra and locus ceruleus. Lewy bodies were seen in those pigmented nuclei, dorsal vagal nucleus, hypothalamus and nucleus basalis of Meynert. No abnormality was found in the intermediolateral nucleus of the spinal cord. This is the first report on a Japanese patient who presented clinically Shy-Drager syndrome and pathologically typical Parkinson's disease. In this patient, from the pharmacological and pathological findings, sympathetic ganglia were supposed to be the responsible lesion for orthostatic hypotension.
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PMID:[An autopsied case of Parkinson's disease manifesting Shy-Drager syndrome]. 130 25

Using a monoclonal antibody directed against the primate nerve growth factor (NGF) receptor, we examined the expression of NGF receptors within neuronal perikarya of normal adult human cerebral cortex (27-98 years old) and individuals with Alzheimer disease (AD). This expression of cortical NGF receptors was compared with that seen in other neurological diseases and normal human development as well as in young and aged nonhuman primates. NGF receptor-containing cortical neurons were not observed in young adults (less than 50 years old) and were observed only infrequently in non-demented elderly individuals (50-80 years old). In contrast, numerous NGF receptor-containing cortical neurons were seen in AD patients of all ages and in one 98-year-old nondemented patient. In advanced age and AD, numerous NGF receptor-positive neurons were located within laminae II-VI of temporal association cortices whereas only a few were seen in the subicular complex, entorhinal cortex, parahippocampal gyrus, and amygdaloid complex. These perikarya appeared healthy, with bipolar, fusiform, or multipolar morphologies and extended varicose dendritic arbors. These neurons failed to express neurofibrillary tangle-bearing material. In contrast to AD, NGF receptor-containing cortical neurons were not observed in Parkinson disease, Pick disease, or Shy-Drager syndrome. The NGF receptor-containing cortical neurons seen in advanced age and AD were similar in morphology to those observed in human fetal cortex. No NGF receptor-containing cortical neurons were observed in young or aged nonhuman primates. These findings suggest that neurons within the human cerebral cortex exhibit plasticity in their expression of NGF receptors in AD and extreme advanced aging.
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PMID:Cortical neurons express nerve growth factor receptors in advanced age and Alzheimer disease. 130 47

Nine cases of multiple system atrophy and 1 case of autosomal dominant olivopontocerebellar atrophy (neuropathological diagnosis) were retrospectively examined for the presence of oligodendroglial inclusions. Clinical diagnosis in the first 9 cases had been: olivopontocerebellar atrophy (3 cases), atypical Parkinson's disease (2 cases), Shy-Drager syndrome (2 cases) and multiple system atrophy (1 case); one of the patients could not be included in any of the above mentioned groups. The oligodendroglial inclusions were argyrophilic and located in the cytoplasm around the nucleus. They were revealed by Bodian's method in all cases of multiple system atrophy. They were not found in the case of autosomal dominant olivopontocerebellar atrophy. They were labelled by anti-ubiquitin antibodies, and were negative with anti-tau antibodies. At electron microscopy, they consisted of rectilinear profiles coated with a fuzzy material (diameter: 20-33 nm); this aspect was compatible with microtubules. Oligodendroglial inclusions were prominent in regions selectively vulnerable in multiple system atrophy (tegmentum pontis, putamen, inferior olives, substantia nigra and cerebellar white matter), even in those areas where neuronal loss or fascicular atrophy were minimal or absent. They were also observed in regions considered to be spared in multiple system atrophy, such as the motor cortex and the corpus callosum. Argyrophilic oligodendroglial inclusions are an early and specific marker of multiple system atrophy. It is suggested that autosomal dominant olivopontocerebellar atrophy lacking oligodendroglial inclusions does not belong to multiple system atrophy.
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PMID:[Oligodendroglial inclusions, a marker of multisystemic atrophies]. 133 74

We studied 18 patients with multiple system atrophy (MSA) by high field strength MRI: 6 striatonigral degeneration (SND), 4 Shy-Drager syndrome (SDS), and 8 olivo-ponto-cerebellar atrophy (OPCA). We also studied 30 Parkinson's disease (PD) and 10 age-matched controls. The diagnoses of SND, SDS, and OPCA were based on criteria after Hirayama et al (1985). Bradykinesia, rigidity, and tremor were assessed with the summed scores of the signs used as the extrapyramidal scores. The mean extrapyramidal scores were not significantly different in patients with SND, SDS, OPCA, and PD. MRI studies were performed on 1.5 tesla MRI unit, using a T2 weighted spin echo pulse sequence (TR2500 ms/TE40 ms). The width of the pars compacta signal in all subjects was measured by the method of Duguid et al (1986). Intensity profiles were made on a straight line perpendicular to the pars compacta through the center of the red nucleus on an image of the midbrain. We measured the width of the valley at half-height between the peaks of intensity representing the red nucleus and the crus cerebri-pars reticulata complex and used this measurement as an index of the width of the pars compacta signal. The mean widths of the pars compacta signal were: 2.8 +/- 0.4 mm (SND), 2.8 +/- 0.7 mm (SDS), 3.6 +/- 0.6 mm (OPCA), 2.7 +/- 0.3 mm (PD), and 4.3 +/- 0.6 mm (control). The mean widths of the pars compacta signal in PD, SND, and SDS were significantly narrower than that in the control group (p < 0.05), while the OPCA group was not significantly narrower.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Magnetic resonance imaging in multiple system atrophy]. 149 Mar 8

The clinical expressions of primary autonomic nervous system failure are more or less numerous, orthostatic hypotension being only one of them. Clinical analysis reveals 3 categories of manifestations: pure progressive dysautonomia, dysautonomia associated with Parkinson's disease, and dysautonomia associated with multiple system atrophy of the nervous system also known as Shy-Drager syndrome. Neuropathological studies show that lesions of the efferent autonomic nervous system (tractus intermediolateralis, sympathetic ganglia) are frequently associated with lesions of the central nervous system the role of which in dysautonomia is still imperfectly known. Lesions of the central nervous system may present as genuine Parkinson's disease with Lew bodies or as multiple systemic atrophy with its two best individualized aspects: striatonigral atrophy and olivopontocerebellar atrophy. These various neurological aspects have their counterpart in biochemical abnormalities, prognosis and response to treatment.
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PMID:[Dysautonomia and multi-systemic atrophy of the nervous system (Shy-Drager's syndrome)]. 153 7


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