Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0233565 (bradykinesia)
 2,352 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The post-mortem examination of the nervous system of a patient with Shy-Drager syndrome successfully treated with levodopa (Sharpe et al, 1972) revealed features of striato-nigral degeneration and amyotrophic lateral sclerosis, a cerebellar system degeneration and a loss of approximately 75% of sympathetic preganglionic neurons. Lewy bodies were not present and no detectable changes were observed in the sympathetic prevertebral ganglia. While the limited and transient beneficial effect of levodopa on the bradykinesia in our case is possibly due to the progressive loss of striatal dopaminergic receptors seen in striatonigral degeneration, we propose that in Shy-Drager syndrome, levodopa therapy benefits orthostatic hypotension because of a suppression of the central depressor action of this drug. This suppression is attributable to functional disconnection of sympathetic ganglia secondary to the loss of preganglionic neurons or to degeneration of central autonomic catecholaminergic systems.
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PMID:Shy-Drager syndrome. Neuropathological correlation and response to levodopa therapy. 96 36

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

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

A 64-year-old carpenter had an unsteady gait, severe dizziness, nocturia, and a loss of erection for more than 4 years. The neurological manifestations consisted of a wide-based ataxic gait, bilateral dysmetria with intentional tremor, staccato speech, rigidity, bradykinesia, and an iris-thinning. There was reproducible orthostatic hypotension. A sweat test revealed severe anhidrosis. Nicotine and methylbenzene sensitivity was absent, whereas norepinephrine infusion test showed a significant elevation of blood pressure. The resting plasma norepinephrine level on recumbency was low and a subnormal surge was noted on standing or exercise. We conclude that the clinical features caused by a degenerative process involving both the central and peripheral autonomic systems, together with atrophy of other systems in this patient, constitute the Shy-Drager syndrome.
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PMID:Abnormal cardiovascular responses to postural changes and pharmacologic agents in a case of Shy-Drager syndrome. 262 36

Shy Drager Syndrome (SDS) is a movement disorder which is often referred to as a parkinson plus syndrome or Multiple System Atrophy (MSA). For patients afflicted with this condition, rigidity and bradykinesia are the primary extrapyramidal symptoms which are present. The "plus" refers to autonomic nervous system dysfunction which leads to much of the disability seen in this disorder. Syncope, urinary incontinence, impotence, constipation, fecal incontinence, cardiac arrythmias as well as other symptoms occur as a result of widespread pathological changes in multiple areas of the central and autonomic nervous system. The goal of this paper is to provide an overview of the pathophysiology, signs and symptoms of and treatment for SDS. Nursing Care of the patient and family coping with Shy Drager Syndrome and the challenges it presents to the Movement Disorder Nurse are discussed. A coordinate, multidisciplinary team approach is suggested.
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PMID:Shy Drager syndrome. 929 81

A previously healthy 60 years old female patient presented with bradykinesia, postural instability and increased rigidity of both upper and lower limbs for 2 years and was diagnosed as Parkinsonism for last years. Later on she developed features of autonomic dysfunction including postural hypotension, hyperhydrosis and urinary incontinence so was diagnosed as Shy-Drager Syndrome. She was treated with fludrocortisone and nefidipine for the management of postural hypotension. Patient developed aspiration pneumonia during oral feeding, despite of ICU management for the same cause patient died of respiratory arrest.
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PMID:Parkisonism with Shy-Drager syndrome--a case report. 1870 Jun 35