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

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

Autonomic failure with Lewy bodies (AF-LB) was first described by Fichefet et al. in 1965, and more than ten cases have been reported to date. AF-LB and Parkinson's disease (PD) share the neuropathological findings characterized by widely distributed Lewy bodies in the central nervous system including the substantia nigra and locus coeruleus. However, clinical manifestations of AF-LB are far different from PD in which autonomic dysfunction, if present, is not a predominant feature. In the present study, clinical features were comparatively analysed in AF-LB and PD to investigate the nosological relation between PD and AF-LB. The subjects were 94 patients with PD and 11 reported cases of AF-LB in the literature. A test of 70 degrees passive head-up tilt was performed upon the patients with PD in our laboratory. Based on the results in tilting test, the patients with PD were divided into two groups; PD-I (69 cases) with an orthostatic fall of systolic blood pressure less than 30 mmHg, and PD-II (25 cases) with a fall of 30 mmHg or more. Autonomic dysfunctions were more extent in PD-II than in PD-I, because the incidences of anhidrosis, impotence, neurogenic bladder and constipation were higher in PD-II. All of the cases of AF-LB were contained in the previous literatures with reasonably full clinical descriptions. Mean age at onset of the disease was 62.1 +/- 8.7 (mean +/- SD) years old in PD-I, 64.5 +/- 7.5 years old in PD-II and 63.9 +/- 9.0 years old in AF-LB, and no significant differences were found among the three groups.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Nosological correlation between autonomic failure with Lewy bodies and Parkinson's disease--comparative assessment of clinical features in both diseases]. 208 26

Shy-Drager syndrome consists of progressive autonomic nervous system failure with Parkinson's disease-like symptoms and orthostatic hypotension. It can also result in airway compromise from bilateral vocal cord paralysis. Fewer than 30 cases of severe bilateral vocal cord paresis or paralysis associated with the Shy-Drager syndrome have been reported in the English literature. We present a case of a 72-year-old man who had a 2-year history of orthostatic hypotension, neurogenic bladder, impotence, anhydrosis, and extremity weakness and paresthesias. Hoarseness and dyspnea with stridor developed as a result of bilateral vocal cord paralysis in the median position and required an emergency tracheotomy. This combination of symptoms resulted in the diagnosis of Shy-Drager syndrome. We present the case along with literature review of bilateral vocal cord paralysis with the Shy-Drager syndrome.
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PMID:Bilateral vocal cord paralysis with Shy-Drager syndrome. 750 34

The clinical features and natural history of 100 patients diagnosed as probable multiple system atrophy (MSA) are described. In all 14 (of 41 deceased) cases who underwent post-mortem examination of the brain, the diagnosis was confirmed pathologically, providing some validation of the clinical diagnostic criteria used. There were 67 men and 33 women. Median age at onset (at time of first reported symptom) was 53 (range 33-76) years. Autonomic symptoms were the initial feature in 41% of the patients, but had subsequently developed in 97% at latest follow-up. The most frequent autonomic symptom in men was impotence, and in women was urinary incontinence. Symptomatic orthostatic hypotension, although present in 68%, was severe in only 15% of patients. Parkinsonism was the initial feature in 46%, but had subsequently developed in 91% of subjects at latest follow-up. It was the predominant motor disorder [striatonigral degeneration (SND) type] in 82% of the patients, and was usually asymmetric (74%). Although akinesia and rigidity predominated, tremor was present at rest in 29% of patients, but in only 9% had a classical pill-rolling parkinsonian rest tremor been recorded. Twenty-nine percent of MSA patients had a good or excellent levodopa response at some stage. However, only 13% maintained this response. Prominent orofacial dyskinesias and dystonias occurred in a quarter of treated patients with MSA. Early onset (before age 49 years) MSA patients tended to have a good levodopa response. Cerebellar symptoms or signs were the only initial feature in 5%. Although subsequently developing in a further 47% of cases, in only 18% was a cerebellar syndrome the only (9%) or predominant (9%) motor disorder [olivopontocerebellar (OPCA) type]. Pyramidal involvement at latest follow-up was noted in 61% of all cases. In a further seven patients the initial features involved more than one system, and one other had presented as a parasomnia. Multiple system atrophy of the OPCA type most commonly presented with gait ataxia. Tremor, pyramidal signs and myoclonus were less common than in MSA of the SND type. Cerebellar signs were present in 42% of patients with MSA of the SND type and parkinsonian signs in 50% of patients with MSA of the OPCA type. Disease progression was faster than in idiopathic Parkinson's disease, so that > 40% of patients were markedly disabled or wheelchair bound within 5 years of onset of motor disturbance. Median survival of the whole group as calculated by Kaplan-Meier analysis was 9.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Clinical features and natural history of multiple system atrophy. An analysis of 100 cases. 792 69

Population aging is continuously increasing in Italy and in the World. Individuals aged 60 years or more are currently 10,500,000 and will be 13,000,000 in 2015. Life quality in geriatric ages includes the maintenance of sexual power: according to recent data (Carrol et al., 1992), 80% of impotence cases are due to organic causes. In addition, the use of drugs can cause impotence. Among them tiazidic diuretics may cause an increase of sexual disturbances. Other drugs with this potential are digitalis, antihypertensive drugs (particularly beta blockers), major and minor tranquillizers, antidepressant, H2 receptor antagonists, antiparkinsonian cholinergic drugs and estrogens employed in the treatment of prostate tumors. Diseases of geriatric age that can alter sexual power are diabetes mellitus, ischemic heart disease for the accompanying depression and for the use of antidepressants; severe hypertension is complicated by impotence in 15% of cases. Among neurological diseases Parkinson's disease and multiple sclerosis can be causes of sexual dysfunctions. Patients on hemodialysis can be impotent, with recent data (Soloh et al 1992) showing that erythropoietin treatment of anemia also improve sexual dysfunctions. Prevention from a geriatric standpoint should be base on action on known risk factor as smoking, alcohol abuse and dislipidemias and with the activation of a close drug vigilance.
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PMID:[Andrologic problems and internal pathology in the elderly]. 825 79

We investigated autonomic function in patients with idiopathic Parkinson's disease (PD) by measuring sympathetic skin response (SSR) and R-R interval variation (RRIV). Sixty-two PD patients and 62 age-matched normal subjects were recruited. Abnormal SSR was noted in nine (14.5%) PD patients, including three in Stage II, three in Stage III, and three in Stage IV, but not in Stage I patients or normal subjects. Four of these nine patients had postural hypotension. Abnormal SSR was correlated with duration of illness and impotence. In PD patients, abnormal SSR may be due to intermediolateral column dysfunction. After logarithmic transformation and age adjustment, 19 (31.6%) of 60 PD patients had abnormal RRIV during rest and deep breathing. Abnormal RRIV was not related to staging or duration of illness. Patients with constipation had significantly lower RRIV, indicating parasympathetic dysfunction. RRIV was not affected by acute or chronic L-dopa treatment. The agreement between RRIV and SSR in PD patients was poor (kappa = -0.07). It appears that abnormal SSR, but not RRIV, may be associated with more autonomic disturbances in PD patients.
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PMID:Sympathetic skin response and R-R interval variation in Parkinson's disease. 847 81

Shy-Drager syndrome (SDS) is a subtype of multiple system atrophy (MSA) with the clinical predominance of autonomic failure. The differential diagnosis between SDS and Parkinson's disease (PD) can sometimes be difficult clinically. The features favoring a clinical diagnosis of SDS are marked orthostatic hypotension, erectile impotence in males, urinary symptoms, nocturnal stridor, rigidity and akinesia without tremors, levodopa unresponsiveness, cerebellar signs, cerebellar atrophy on brain CT scans and MRI.
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PMID:[Shy-Drager syndrome and multiple system atrophy]. 901 36

Penile erections were regularly induced by intermittent subcutaneous injections of apomorphine in five patients with Parkinson's disease (PD) complicated by motor fluctuations. Four of the patients reported erectile dysfunction before beginning apomorphine and two of these report a significant improvement in their sexual function resulting from apomorphine use. Animal studies suggest central D2-type dopamine receptor stimulation and oxytocin release from the paraventricular nucleus of the hypothalamus mediate the effect. Erections reported with other dopamine agonists and levodopa are probably mediated by the same mechanism. Apomorphine-induced erections in PD are probably more common than previously thought. The benefit of apomorphine on sexual function in some patients suggests a possible role in the treatment of impotence in PD.
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PMID:Apomorphine-induced penile erections in Parkinson's disease. 1043 14

Modern molecular biology has revealed vast numbers of large and complex proteins and genes that regulate body function. By contrast, discoveries over the past ten years indicate that crucial features of neuronal communication, blood vessel modulation and immune response are mediated by a remarkably simple chemical, nitric oxide (NO). Endogenous NO is generated from arginine by a family of three distinct calmodulin- dependent NO synthase (NOS) enzymes. NOS from endothelial cells (eNOS) and neurons (nNOS) are both constitutively expressed enzymes, whose activities are stimulated by increases in intracellular calcium. Immune functions for NO are mediated by a calcium-independent inducible NOS (iNOS). Expression of iNOS protein requires transcriptional activation, which is mediated by specific combinations of cytokines. All three NOS use NADPH as an electron donor and employ five enzyme cofactors to catalyze a five-electron oxidation of arginine to NO with stoichiometric formation of citrulline. The highest levels of NO throughout the body are found in neurons, where NO functions as a unique messenger molecule. In the autonomic nervous system NO functions NO functions as a major non-adrenergic non-cholinergic (NANC) neurotransmitter. This NANC pathway plays a particularly important role in producing relaxation of smooth muscle in the cerebral circulation and the gastrointestinal, urogenital and respiratory tracts. Dysregulation of NOS activity in autonomic nerves plays a major role in diverse pathophysiological conditions including migraine headache, hypertrophic pyloric stenosis and male impotence. In the brain, NO functions as a neuromodulator and appears to mediate aspects of learning and memory. Although endogenous NO was originally appreciated as a mediator of smooth muscle relaxation, NO also plays a major role in skeletal muscle. Physiologically muscle-derived NO regulates skeletal muscle contractility and exercise-induced glucose uptake. nNOS occurs at the plasma membrane of skeletal muscle which facilitates diffusion of NO to the vasculature to regulate muscle perfusion. nNOS protein occurs in the dystrophin complex in skeletal muscle and NO may therefore participate in the pathophysiology of muscular dystrophy. NO signalling in excitable tissues requires rapid and controlled delivery of NO to specific cellular targets. This tight control of NO signalling is largely regulated at the level of NO biosynthesis. Acute control of nNOS activity is mediated by allosteric enzyme regulation, by posttranslational modification and by subcellular targeting of the enzyme. nNOS protein levels are also dynamically regulated by changes in gene transcription, and this affords long-lasting changes in tissue NO levels. While NO normally functions as a physiological neuronal mediator, excess production of NO mediates brain injury. Overactivation of glutamate receptors associated with cerebral ischemia and other excitotoxic processes results in massive release of NO. As a free radical, NO is inherently reactive and mediates cellular toxicity by damaging critical metabolic enzymes and by reacting with superoxide to form an even more potent oxidant, peroxynitrite. Through these mechanisms, NO appears to play a major role in the pathophysiology of stroke, Parkinson's disease, Huntington's disease and amyotrophic lateral sclerosis.
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PMID:Endogenous nitric oxide synthesis: biological functions and pathophysiology. 1063 Jun 82


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