[RADIOCINEMATOGRAPHY OF ESOPHAGEAL DYSKINESIAS. A NEW APPROACH].
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UNLABELLED: Degenerative process in Parkinson's disease affects substantia nigra and other central structures of an extrapyramidal system but it can also affect central and peripheral autonomic centres. One of the most frequent late complications in levodopa therapy is a wearing off phenomenon. We present a patient treated for Parkinson's disease in whom during the period of levodopa wearing off we observed a paroxysmal abdominalgia apart from other features of a typical movement disorders like: increasing rigidity, gait disturbances and tremor. Abdominalgia consisted of stomach cramps, with variable localization in epi-, meso- and hypogastrium. Rectal tenesmus was also present. The patient was treated with analgesics, spasmolytics and carminative drugs with no effect. Abdominal pains regressed after an intake of the next levodopa dose. The patient presented with other features of a gastrointestinal tract autonomic system dysfunction like: chronic constipation, preterm satiety resulting in food intake reduction and a decrease in body weight. There was no organic lesions of the gastrointestinal system that could explain such disturbances. Pharmacologic treatment modification (more frequent levodopa dosage, additional dopamine agonist) resulted in some improvement. CONCLUSION: It is possible that the abdominal pains could be a clinical manifestation of a digestive tract dyskinesias, occurring during
Most patients with Parkinson's disease experience autonomic dysfunction at some point in the course of their disease. In contrast to autonomic dysregulation in multisystem atrophy, they are less severe, and they frequently cause troublesome symptoms only in advanced stages of the disease. The quality of life is nevertheless substantially restricted. Cardiovascular, gastrointestinal and urogenital autonomic dysfunction is predominant. Appropriate diagnosis and treatment can greatly benefit the patient. An interdisciplinary approach is desirable in most cases.
Neurotoxicity is a well-recognized and commonly observed side effect associated with the use of vincristine sulfate in cancer chemotherapy. The clinical manifestations of vincristine neuropathy cover a wide spectrum of peripheral neurologic dysfunctions that have been described to be reversible and cumulative in most instances (1, 2). Paresthesias, loss of tendon reflexes, and progressive weakness are the most common clinical features (3, 4). Sensory impairment, cranial nerve palsies, gastrointestinal disturbances, and autonomic dysfunctions including atonic bladder, impotence, and orthostatic hypotension may occur (5). Acute CNS complications, usually presenting as generalized seizures, are extremely rare and only a few cases have been reported which were without underlying biochemical or structural abnormalities (1, 5-9). We describe the case of a woman with multiple myeloma, who developed fulminant encephalopathy following 4 days of continuous vincristine, adriamycin, and day 1-4 pulse dexamethasone (VAD) combination therapy.
Following acute thermal injury to rats produced by scalding water, there was marked elevation of a number of plasma enzyme activities, including GOT, GPT, and 5'-nucleotidase, suggesting hepatic dysfunction. Changes in plasma enzyme activities were observed within minutes following application of acute burn trauma, and remained elevated for at least one month. The magnitude of the elevations of the plasma enzyme activities was dependent upon the length of time the acute burn trauma was applied to the skin and/or the percentage of skin surface area burned. These changes in plasma enzyme activity correlated with histologic examination of the hepatic tissue, indicating single cell necrosis. These data suggest that acute burn trauma to rats is associated with altered hepatic function.
Patients with functional gastrointestinal disorders may have visceral sensory dysfunction so that physiological stimuli induce their symptoms. The clinical significance of altered perception-that is, its relation to clinical symptoms-remains unclear. Data indicate that sensory dysfunction is associated with altered reflex activity. Hence evidence of combined sensory-reflex dysfunction as a common pathophysiological mechanism in various functional gastrointestinal disorders would suggest that they are different forms of the same process. Altered reflex activity and altered conscious perception of gastrointestinal stimuli may combine to differing degrees, and their interaction may produce clinical symptoms.
This update focuses on swallowing disorders and sphincter of Oddi dysfunction. Anatomy and physiology of swallowing are described, as are the signs, symptoms, and etiology of swallowing disorders. The imaging of these patients, particularly with videofluoroscopic swallowing study and fiberoptic endoscopic examination of swallowing, is then discussed. Sphincter of Oddi dysfunction as a cause of postcholecystectomy syndrome as well as its classification is described. This is followed by an explanation of the roles of fatty meal sonography and hepatobiliary scintigraphy in patients with sphincter of Oddi dysfunction, particularly type II and type III.
PURPOSE OF REVIEW: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive loss of motor neurones, but it is increasingly recognized to be a more disseminated disease. The autonomic nervous system may also be involved. Here we review the literature with specific emphasis on autonomic functions in ALS. RECENT STUDIES: Ample evidence exists for subclinical dysfunction of cardiovascular, sudomotor, gastrointestinal, salivary and lacrimal regulation, even in early ALS cases. Autonomic disturbances may lead to circulatory collapse or sudden death in respirator dependent patients. Several studies suggest the existence of sympathetic hyperactivity in ALS. We discuss some possible pathophysiological mechanisms of the subtle abnormalities and some clinical and treatment implications. SUMMARY: The wide range of autonomic involvement, together with results suggesting cognitive and extrapyramidal dysfunction, supports the view that ALS is a multisystem degenerative disease.
Gastrointestinal symptoms occur in a variety of neuromuscular diseases that may affect the neural access from the brain down to the peripheral nerve. This review addresses the clinical diagnosis of the gastrointestinal manifestations that occur in neuromuscular diseases. Prototypic examples are diabetic autonomic neuropathy, parkinsonism, and scleroderma. However, there is increasing recognition of the importance of disorders of the development of the enteric nervous system. For example, disturbances of the interstitial cells of Cajal (the pacemakers of the intestine) result in neuromuscular disorders and metabolic disorders of oxidative phosphorylation manifest as mitochondrial cytopathies that affect all muscle including the gut. The reviewer will discuss the use of specialized gastrointestinal measurements to detect dysfunction of different regions of the gut, including transit, volumetric measurements of the stomach, and manometry. The principles of management are treatment of the neurological disease itself, support of hydration and nutrition, symptom relief, suppression of bacterial overgrowth, correction of dysmotility with selective agonists and antagonists of neurotransmitter receptors and neurotrophic factors, and, rarely, surgery.
Cyclooxygenases (COX) are the target of non-steroidal anti-inflammatory drugs (NSAIDs) which exert their therapeutic effect by blocking COX's capacity to metabolize arachidonate to a series of biologically active fatty acids, designated prostaglandins. NSAID use is associated with two major tonicities: gastrointestinal bleeding and renal dysfunction. In the setting of significant physiologic stress, renal function becomes dependent upon prostaglandins and NSAID use may be associated with acute deterioration of renal function, including development of sodium retention, edema, hypertension, hyperkalemia, and or papillary necrosis. Two isoforms, COX1 and COX2, have been identified. They are products of distinct genes and their expression is under different regulatory control. Both COX1 and COX2 are highly expressed in the kidney and both are inhibited by conventional NSAIDs. Accumulating data using recently developed selective COX2 inhibitors suggest that while these agents spare the gastrointestinal tract they have similar renal effects as non-selective NSAIDs. Therefore, caution should be taken when prescribing selective COX2 inhibitor to patients, especially to patients with predisposed physiologic stress.
The ultrastructural finding of abnormal muscle mitochondria has been reported in various conditions, but mostly in association with the clinical picture of ophthalmoplegia, and in cases of "floppy infant" syndrome. In the case herein reported, the mitochondrial abnormalities were found in the muscle biopsy of a 49-year-old man suffering from a late onset proximal myopathy; he was affected also by polyneuropathy, subclinical thyroid dysfunction, disturbances of heart conduction, and unilateral gynaecomastia. The association of abnormal muscle mitochondria and late onset myopathy without involvement of the extraocular muscles has been reported in a very few cases. It is not possible, at present, to state that these cases represent a nosological entity; the existence of an underlying biochemical defect, accounting for the mitochondrial abnormalities, could be suspected, but it seems more probable that these changes are non-specific features of muscular damage, possibly related to the stage and the degree of the process. In this view, the coexistence of neurogenic damage, gastrointestinal malabsorption, and thyroid dysfunction, could play an additional role in the case herein described. Finally, the coexisting findings of cardiac, endocrine, and neuropathic damage are discussed with regard to the Kearns-Sayre syndrome, which also associates mitochondrial myopathy and multisystemic involvement.
Nonsteroidal anti-inflammatory drugs (NSAIDs) are widely used in the therapy of rheumatic diseases, especially in older patients. The toxicity profile of NSAIDs includes gastrointestinal (GI) toxicity, renal dysfunction and hepatic disease. Altered drug pharmacology in older patients may be a factor in their increased risk for drug toxicity. Elderly patients appear to be at greatest risk for symptomatic GI toxicity, including ulceration and even major GI bleeding. Central nervous system toxicity, characterized by dizziness, headaches, mood alteration and confusion, and renal dysfunction have also been reported to occur more commonly among elderly patients. Hepatic dysfunction is a rare NSAID-induced toxicity, but older patients are often at greatest risk for serious hepatic disease. Understanding the patient's underlying physiologic condition, concomitant drug therapy and the kinetics of the NSAID being used is critical to the safe administration of these agents to elderly individuals.
Spontaneous bacterial peritonitis (SBP) is an ascitic fluid infection as a complication of end stage liver disease. The outcome is related to the severity of hepatorenal function, gastrointestinal bleeding, and many others; however it is not well known whether the infection acquisition sites have an effect on the prognosis of SBP. In order to identify the prognostic significance of the acquisition sites, we studied 106 patients who were diagnosed as culture positive SBP between October 1998 and August 2003. Thirty-two episodes were nosocomial and 74 were community acquired. Gram-negative bacilli such as Escherichia coli were dominant in both of the nosocomial and community-acquired SBPs. Despite significantly higher resistance to cefotaxime in nosocomial isolates compared to community-acquired isolates (77.8% vs. 13.6%, p=0.001), no difference was found regarding short or long term prognosis. Infection acquisition sites were not related to short or long term prognosis either. Shock, gastrointestinal bleeding and renal dysfunction were related to short term prognosis. Only Child-Pugh class C was identified as an independent prognostic factor of long-term survival.
A clinical epidemiologic study was conducted to determine the long-term health effects of workplace exposure to the process of manufacturing the herbicide (2,4,5-trichlorophenoxy)acetic acid including contaminants such as 2,3,7,8-tetrachlorodibenzo-rho-dioxin. The population consisted of two cohorts: 204 clearly exposed and 163 not exposed. Among the exposed, clinical evidence of chloracne persisted in 55.7%. None of the not exposed experienced chloracne development. An association was found between the persistence of chloracne and the presence and severity of actinic elastosis of the skin. There is an association between exposure and the history of gastrointestinal tract ulcer. Pulmonary function values among those who were exposed and who currently smoked were lower than those who were not exposed and who currently smoked. The data assembled in the study indicate no evidence of increased risk for cardiovascular disease, hepatic disease, renal damage, or central or peripheral nervous system problems.
BACKGROUND: The presence of autonomic neuropathy impairs the quality of life (orthostatic hypotension, impotence, gastroparesis) or endangers the life of diabetics (sudden death, unawareness of hypoglycemia). The purpose of the investigation was: 1. To assess the presence of the autonomic neuropathy of the cardiovascular and the gastrointestinal systems and their mutual relationship. 2. To assess the relationship of found autonomic neuropathy and the subjective symptoms which are typical to affected particular systems (cardiovascular, gastrointestinal, genitourinary, sudomotor systems and the syndrome of unawareness of hypoglycemia). METHODS AND RESULTS: The group comprised of 25 type 1 diabetic patients (12 women and 13 men) mean age 40.5 +/- 11.6 (range 21-57 years) with a mean duration of diabetes of 17.8 +/- 7.9 (range 4-35 years), treated with intensified insulin regimens. The cardiovascular autonomic neuropathy was automatically examined by the VariaPulse TF 3 computer system. Scintigraphy was used to investigate the gastric emptying time of 99mTc labelled rice. The information about the subjective symptoms we collected from the questionnaire. For statistic analysis we used Spearmen correlations and ANOVA. RESULTS: 1. A statistically significant correlation was found between the presence of the autonomic neuropathy of cardiovascular and gastrointestinal systems (r = 0.634, p < 0.0007). 2. We didn't find any relation among the cardiovascular autonomic neuropathy and the subjective symptoms of cardiovascular system, respectively the gastrointestinal neuropathy (impair gastric emptying) and the subjective symptoms of gastrointestinal system. We found a significant correlation between cardiovascular and gastrointestinal neuropathy and erectile dysfunction (r = 0.48, p < 0.0078), (r = 0.42, p < 0.0388) and with the syndrome of hypoglycemia unawareness (r = 0.49, p < 0.0057), (r = 0.52, p < 0.0075). CONCLUSIONS: The evidence of the cardiovascular autonomic neuropathy is warning signal for the affected autonomic neuropathy in other systems which are more complicated for diagnostic. The subjective symptoms don't correlate with the presence of the visceral neuropathy.
A total of 9928 patients taking cimetidine and 9351 controls were included in a post-marketing drug surveillance study in Glasgow, Nottingham, Oxford and Portsmouth; 98.8 per cent of the takers and 97.7 per cent of the controls were successfully followed up for at least one year during which hospital visits and deaths were recorded. Methods of identification of subjects and 12-month mortality results have been reported previously. A general analysis of the morbidity experienced by these patients during the study year is presented here. Thirty-nine per cent of takers and 21 per cent of controls were seen at outpatient clinics, and 18 per cent of takers and 8 per cent of controls were admitted to hospital; 15 325 individual diagnoses in takers and 5002 diagnoses in controls were reviewed. An association with cimetidine treatment was found, as expected, for gastrointestinal diseases. Weaker associations were found for haematological disorders, some tumours, infections, disorders of the locomotor system and respiratory diseases. Detailed examination revealed that these were mainly due to confounding from several sources, for example, from the underlying cause of the dyspepsia which resulted in cimetidine use, from the higher level of physician contact in cimetidine takers, and smoking. The scheme successfully detected and quantified some already known adverse effects of cimetidine and did not detect any new effects. It is concluded that this method of collecting information is feasible and useful, but several interpretive pitfalls arise, some of which can be avoided by careful analysis. No evidence of any major unrecognised risk of cimetidine treatment emerged from the study.
In several gastroenterological diseases andrological anomalies have become known. From this point of view up to now the liver cirrhosis has been examined most frequently. In patients with cirrhosis apparently the two functions of the testicles are disturbed. On the one hand, a decreased or missing fertility is to be assumed, on the other hand, a cirrhosis does not always exclude the procreative capacity. The hormone analyses plead for the fact that the hypogonadism might rather be conditioned testicularly, in which case the direct toxicity of alcohol may be of importance. In haemochromatosis the hypogonadism develops by a combined mechanism. The cystic fibrosis of the pancreas is practically always associated with a male infertility. In Crohn's disease a disturbance of the spermatogenesis is observed. Even the salazo-sulphapyridine therapy is accompanied by unfavourable influences on the spermiogramme. Cimetidine used in the ulcer therapy shows a certain antiandrogenic effect and after a longer time may evoke impotence and other undesirable andrological side-effects which we, however, did not realize in 4 weeks treatment periods.
Cantharidin, known popularly as Spanish fly, has been used for millennia as a sexual stimulant. The chemical is derived from blister beetles and is notable for its vesicant properties. While most commonly available preparations of Spanish fly contain cantharidin in negligible amounts, if at all, the chemical is available illicitly in concentrations capable of causing severe toxicity. Symptoms of cantharidin poisoning include burning of the mouth, dysphagia, nausea, hematemesis, gross hematuria, and dysuria. Mucosal erosion and hemorrhage is seen in the upper gastrointestinal (GI) tract. Renal dysfunction is common and related to acute tubular necrosis and glomerular destruction. Priapism, seizures, and cardiac abnormalities are less commonly seen. We report four cases of cantharidin poisoning presenting to our emergency department with complaints of dysuria and dark urine. Three patients had abdominal pain, one had flank pain, and the one woman had vaginal bleeding. Three had hematuria and two had occult rectal bleeding. Low-grade disseminated intravascular coagulation, not previously associated with cantharidin poisoning, was noted in two patients. Management of cantharidin poisoning is supportive. Given the widespread availability of Spanish fly, its reputation as an aphrodisiac, and the fact that ingestion is frequently unwitting, cantharidin poisoning may be a more common cause of morbidity than is generally recognized. Cantharidin poisoning should be suspected in any patient presenting with unexplained hematuria or with GI hemorrhage associated with diffuse injury of the upper GI tract.