[The Melkersson-Rosenthal syndrome. Clinical aspects, nosology and therapy].
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The "anorectic syndrome" of patients with malignant diseases is a symptom complex consisting of loss of appetite, aversion against food and disturbances of taste and smell. Anorexia is defined as a decrease of nourishment below 80 % of the usual food intake. Occurrence of anorexia depends on the type and location of the tumor and on tumor stage. About 40 % of tumor patients suffer from anorexia already at the time of diagnosis. The highest prevalence is seen in advanced tumor stages the etiology of anorexia is multifactorial. The causes mentioned include food aversion, disturbances of taste, gastrointestinal disorders, pain, treatment sequelas, consequences of metabolic disturbances, effects and interactions of cytokines, neuropeptides and neurotransmitters, reduced exercise as well as psychological factors and conditioned aversions. Treatment options of anorexia consist of nutritional measures like "preference guided nutrition" considering the patients individual aversions and preferences. Drugs recommended for treatment are procinetic agents, corticosteroids, gestagens or cannabinoids
A 35-year-old man, previously healthy except for a grade 1 goitre, sustained a spontaneous left pneumothorax treated with a Bülau drain. When the left pneumothorax recurred 2 months later a left thoracotomy was performed. Two bullae at the lung apex were resected and a pleurodesis performed. After the operation the patient noted hypaesthesia of the dorsum of the left upper arm, mild ptosis of the left eyelid as well as reduced sweat secretion over the left half of the face and the left rib cage. The hypaesthesia improved, but the sympathetic nerve deficits remained. There were no other neurological signs. 9 months later, within one minute of eating a sour apple, the patient developed severe sweating over the left half of the face and the left chest. The reaction was confirmed by infra-red thermography which proved that the skin temperature in the sweating region had fallen to 3 degrees C. The likely cause of localized gustatory sweating is intra-operative damage of the stellate ganglion or its preganglionic nerve connections. Treatment is limited to avoidance of the precipitating gustatory stimulus.
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BACKGROUND: Oral mucositis is a complication of intensive chemotherapy and radiotherapy with no effective treatment. We tested the ability of palifermin (recombinant human keratinocyte growth factor) to decrease oral mucosal injury induced by cytotoxic therapy. METHODS: This double-blind study compared the effect of palifermin with that of a placebo on the development of oral mucositis in 212 patients with hematologic cancers; 106 patients received palifermin (60 microg per kilogram of body weight per day) and 106 received a placebo intravenously for three consecutive days immediately before the initiation of conditioning therapy (fractionated total-body irradiation plus high-dose chemotherapy) and after autologous hematopoietic stem-cell transplantation. Oral mucositis was evaluated daily for 28 days after transplantation. RESULTS: The incidence of oral mucositis of World Health Organization (WHO) grade 3 or 4 was 63 percent in the palifermin group and 98 percent in the placebo group (P<0.001). Among patients with this degree of mucositis, the median duration of mucositis was 6 days (range, 1 to 22) in the palifermin group and 9 days (range, 1 to 27) in the placebo group. Among all patients, regardless of the occurrence of mucositis, the median duration of oral mucositis of WHO grade 3 or 4 was 3 days (range, 0 to 22) in the palifermin group and 9 days (range, 0 to 27) in the placebo group (P<0.001). As compared with placebo, palifermin was associated with significant reductions in the incidence of grade 4 oral mucositis (20 percent vs. 62 percent, P<0.001), patient-reported soreness of the mouth and throat (area-under-the-curve score, 29.0 [range, 0 to 98] vs. 46.8 [range, 0 to 110]; P<0.001), the use of opioid analgesics (median, 212 mg of morphine equivalents [range, 0 to 9418] vs. 535 mg of morphine equivalents [range, 0 to 9418], P<0.001), and the incidence of use of total parenteral nutrition (31 percent vs. 55 percent, P<0.001). Adverse events, mainly rash, pruritus, erythema, mouth and tongue disorders, and taste alteration, were mild to moderate in severity and were transient. CONCLUSIONS: Palifermin reduced the duration and severity of oral mucositis after intensive chemotherapy and radiotherapy for hematologic cancers.
OBJECTIVE: The Oral Health Science Institute at the University of Pittsburgh has completed a cross-sectional epidemiologic study of 406 subjects with type 1 diabetes and 268 control subjects without diabetes that assessed the associations between oral health and diabetes. This report describes the prevalence of dry-mouth symptoms (xerostomia), the prevalence of hyposalivation in this population, and the possible interrelationships between salivary dysfunction and diabetic complications. STUDY DESIGN: The subjects with diabetes were participants in the Pittsburgh Epidemiology of Diabetes Complications study who were enrolled in an oral health substudy. Control subjects were spouses or best friends of participants or persons recruited from the community through advertisements in local newspapers. Assessments of salivary function included self-reported xerostomia measures and quantification of resting and stimulated whole saliva flow rates. RESULTS: Subjects with diabetes reported symptoms of dry mouth more frequently than did control subjects. Salivary flow rates were also impaired in the subjects with diabetes. Regression models of potential predictor variables were created for the 3 self-reported xerostomia measures and 4 salivary flow rate variables. Of the medical diabetic complications studied (ie, retinopathy, peripheral and autonomic neuropathy, nephropathy, and peripheral vascular disease), only neuropathy was found to be associated with xerostomia and decreased salivary flow measures. A report of dry-mouth symptoms was associated with current use of cigarettes, dysgeusia (report of a bad taste), and more frequent snacking behavior. Xerogenic medications and elevated fasting blood glucose concentrations were significantly associated with decreased salivary flow. Resting salivary flow rates less than 0.01 mL/min were associated with a slightly higher prevalence of dental caries. Subjects who reported higher levels of alcohol consumption were less likely to have lower rates of stimulated salivary flow. CONCLUSIONS: Subjects with type 1 diabetes who had developed neuropathy more often reported symptoms of dry mouth as well as symptoms of decreased salivary flow rates. Because of the importance of saliva in the maintenance and the preservation of oral health, management of oral diseases in diabetic patients should include a comprehensive evaluation of salivary function.
Biochemical characteristics of gustin, the major zinc protein in human parotid saliva, are similar whether the protein is isolated from subjects with normal taste acuity or from patients with hypogeusia (who may have a little as 1/5th as much parotid saliva gustin as normal subjects do). Zinc concentration in fraction II of parotid saliva, the fraction in which gustin is found on Sephadex G-150 or Sephacryl S-200 column chromatography, is proportional to the gustin content of saliva and is decreased in patients with lower than normal total parotid saliva zinc. The quantity and spectrophotometric indices of all other protein fractions isolated from patients by these column chromatographic techniques did not differ from those of normals. One patient with proven hypogeusia and low concentrations of zinc in total parotid saliva and fraction II, after 9 days of treatment with exogenous zinc, showed a 150% increase in fraction II zinc and a concomitant increase in apparent gustin levels; these changes preceded the return of normal taste function. These data demonstrate that zinc treatment can affect both taste and gustin concentrations in hypogeusia.
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Previous studies evaluating short-term itraconazole and terbinafine therapy for onychomycosis have varied in protocol and size; this double-blind study enabled a large-scale, standardized, direct comparison. Patients with toenail onychomycosis were randomized to itraconazole 200 mg daily (n = 146) or terbinafine 250 mg daily (n = 146) for 12 weeks, with a 36-week follow-up. Mycological cure rates at the follow-up end-point were significantly equivalent (61% with itraconazole vs. 67% with terbinafine). A similar proportion of patients in each group experienced adverse events during treatment (itraconazole, 22%; terbinafine, 23%). More patients receiving terbinafine stopped treatment permanently because of treatment-related adverse events (8% vs. 1%).
The course of recovery from taste receptor disturbance was studied in 119 patients with moderate-to-severe taste receptor disturbance that was cured or improved with zinc therapy. Taste receptor disturbance was idiopathic in 45 patients, drug-induced in 38 and due to zinc deficiency in 36. Recovery of taste, evaluated by filter paper disk testing and electrogustometry, followed 1 of 3 patterns: (i) in 54 (45.4%) of the 119 cases, taste improved simultaneously in the anterior (innervated by the chorda tympani nerve) and posterior (innervated by the glossopharyngeal nerve) portions of the tongue; (ii) in 53 (44.5%) of the cases, taste improved in the posterior portion first; and (iii) in 12 (10.1%) of the cases, taste improved in the anterior portion first. Zinc therapy was more effective in patients with the "posterior" pattern of recovery, and these patients also recovered the ability to sense sweet and bitter tastes earlier than other tastes. These results indicate that recovery of taste begins on the posterior portion of the tongue, which has an abundance of taste buds. The results of electrogustometry were not helpful in assessing recovery from taste disturbance, but testing for taste using the filter paper disk method on the posterior portion of the tongue was useful for identifying the onset of recovery.
Taste disturbance may result from injury to the lingual branch of the glossopharyngeal nerve (LBGN) during tonsillectomy. Because an understanding of the anatomy of this nerve is required in order to avoid injuring it, a gross, histologic anatomic study was undertaken of the topographical relationship between the LBGN and the muscle layer of the palatine tonsillar bed. Evaluation of 107 sides of 83 Japanese adult cadavers (aged 27-88 years) confirmed that the muscular composition and lamination of the tonsillar bed do not change with age or pathological conditions such as inflammation. In about a quarter (23.4%) of cases, the LBGN traveled inferior to the styloglossus muscle and lateral to the superior constrictor pharyngeal muscle over its whole course to the base of the tongue, so that the palatine tonsil was clearly separated from the LBGN. In 55.1% of cases, however, the muscle lining of the tonsillar bed was discontinuous and thin muscle bundles, derived from the stylopharyngeus, palatopharyngeus or superior constrictor pharyngeal muscle, partially covered the tonsillar capsule externally. Moreover, in 21.5% of cases the LBGN was firmly adherent to the tonsillar capsule, due to the complete absence of muscles lining the tonsillar bed. In these cases, and also probably in a similar percentage of patients undergoing tonsillectomy, taste disturbance may occur on removal of the hypertrophic tonsillar capsule. Therefore, minimal disturbance of the tonsillar bed is recommended in all cases of tonsillectomy.
We tested sensory and secretomotor function of the greater petrosal nerve (GPN) by means of electrogustometry (EGM) of the soft palate and Schirmer's tear test in 115 patients (59 males, 56 females) with acute peripheral facial paralysis. Facial paralysis was caused by Bell's palsy in 78 cases, Ramsay Hunt syndrome in 27 cases and zoster sine herpetic lesions in 10. All patients had dysfunction of the stapedial nerve. An electrogustometer was used to test taste (GPN sensory function), and elevation of the threshold by > 6 dB on the affected side was considered abnormal. Schirmer's test was used to evaluate lacrimal (GPN secretomotor) function, which was considered abnormal when tear secretion on the affected side was < 50% of secretion on the non-affected side. Of the 78 patients with Bell's palsy, 28.2% had altered taste on the soft palate (sensory dysfunction) and 10.3% had lacrimal dysfunction, indicating that EGM of the soft palate is more sensitive than Schirmer's test for identifying dysfunction of the GPN in patients with facial paralysis due to Bell's palsy. Of the total of 115 patients, 32 (28%) had taste dysfunction and 9 (28.1%) of these 32 patients also had lacrimal dysfunction. This finding indicates that facial paralysis has different effects on the sensory and secretory nerve fibers of the GPN. The results of Schirmer's test were more closely related to the severity of, and prognosis for, facial paralysis than the results of EGM.
Drug-induced change in taste is the second most common cause of taste disturbance among our patients, accounting for approximately 25% of cases. About 170 drugs have been associated with taste disturbances, either when used alone or in combinations. We studied the zinc-chelating capability of 20 drugs associated with taste disturbance, using the pH titration test, DC polarography, spectrophotometry and tests of intestinal absorption of zinc in the presence of these drugs. The results of these analyses and our review of the literature indicate that there are a number of possible mechanisms for drug-related taste disturbance and that zinc plays a key role.
Changes in gustatory sense were investigated in 97 pregnant women and in 30 healthy, non-pregnant women who served as controls. All 97 pregnant women completed a questionnaire regarding taste changes and 32 of them underwent serial tests of gustatory function, including electrogustometry and testing with filter paper disks placed over the areas of the chorda tympani nerve and glossopharyngeal nerve. All gustatory testing was performed by the same person. In addition, serum levels of trace elements were measured in 72 of the pregnant women. Of the 97 pregnant women, 90 (92.8%) reported in the questionnaire that they had experienced some type of change in taste during pregnancy, usually a change in sour taste (59 women; 65.6%). Pregnant women had higher gustatory thresholds than non-pregnant women, with an especially marked decrease in gustatory function being noticed in the first trimester. Although serum zinc levels decreased in pregnant women between the second and third trimesters, zinc levels were in the normal range in the early stage of pregnancy. Thus, it is difficult to explain dysgeusia in the early stage of pregnancy as being associated with a deficiency of zinc. The decrease in gustatory function during the first trimester is considered to be due to the notable changes in secretion of hormones that occur during this stage.
Protein-calorie malnutrition, vitamin and other deficiencies, and weight loss frequently develop in cancer patients. Although there is no evidence that aggressive nutritional management prolongs survival, it may improve the quality of life. Efforts should be made to maintain adequate daily caloric intake with appropriate food selection and with control of complications interfering with nutrition. In selected patients, intravenous hyperalimentation can provide adequate nutrition during potentially effective chemotherapy or radiotherapy. Elemental diets also may be a source of complete or supplemental nutrition. Further experience with both approaches will help to clarify their role in the nutritional management of the patient with advanced cancer.
Findings from 441 patient evaluations performed at the Taste and Smell Clinic of the Connecticut Chemosensory Clinical Research Center are presented. Taste and smell dysfunction was the chief complaint in all patients. The diagnostic approach included tests of taste and smell function; history taking; physical, neurologic, otorhinolaryngologic, and nutritional examinations; and clinical laboratory screening tests. Results indicate that the most common chemosensory dysfunction is probably olfactory: 86% of patients in this series had measurable loss of smell function. The most common cause of olfactory deficit was nasal and/or sinus disease (30% of patients), followed by idiopathic conditions (26%) and prior upper respiratory infection (19%). Assessment of individual components of the evaluation suggests that a total circulating eosinophil count may be useful as a screening test for nasal and/or sinus disease among patients whose chief complaint is chemosensory dysfunction.
Following traumatic brain injury (TBI) the complete disappearance of food aversions was observed in the cases of two patients. In one of the cases, a young female, this change in her food aversion was manifested several months after the TBI, from the time when she was able to eat normally. The other patient, a young man, exhibited the disappearance of his food aversion immediately after recovery from his unconscious state following TBI. These results indicate that the disappearance of food aversions was a consequence of TBI.
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