Tonic pupil,areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature

Summary Two patients are described with the triad of tonic pupil, hyporeflexia and segmental anhidrosis (Ross syndrome). Only 18 cases of this syndrome have been reported in the literature so far. While tonic pupil and reduced sweating can be attributed to the affection of postganglionic cholinergic parasympathetic and sympathetic fibres projecting to the iris and sweat glands, respectively, the pathogenesis of diminished or lost tendon jerks remains obscure. To identify the characteristic clinical features, the previous cases of Ross syndrome are reviewed. Recent evidence of subclinical disturbances of sweating in most patients with Adie's syndrome, i.e. tonic pupil and areflexia, casts doubt on the nosological concept of Ross syndrome as a distinct clinical entity.     

Two-year double-blind trial of a monomethoxy polyethylene glycol (mPEG) modified grass pollen extract at different dose levels.

The aim of this study was to compare the efficacy and safety of a monomethoxypolyethylene glycol (mPEG) modified grass pollen mix allergen preparation (mPEG-gm) and a partly purified grass pollen mix allergen preparation (gm) in hyposensitization (HS), evaluating both products at two dose levels. Thirty adult patients with allergic rhinoconjunctivitis were allocated into two treatment groups based on their sensitivity to conjunctival provocation tests (CPT). Treatment was given in a double-blind manner. The starting dose was 20 BU and was approximately doubled weekly up to 20,000 BU the first year and 120,000 BU the second year. Skin testing and CPT were performed before treatment and at each dose level. All patients reached 20,000 BU the first year. Twenty-five patients continued the second year. Twenty-one of those reached 120,000 BU (9/12 on mPEG-gm and 12/13 on gm). The frequency of general side effects was reduced by about 50% with the mPEG grass mix compared with native grass mix. A significant improvement in the conjunctival sensitivity was found in both treatment groups the second year (120,000 BU) but not the first year (20,000 BU). Seventy-eight percent of the patients in the gm group and 50% in the mPEG-gm group improved by CPT (not statistically significant). The skin sensitivity was reduced after 1 year at low dose in 69% of the gm-treated patients and 33% of the mPEG treated patients. After the second year at high dose levels, the skin sensitivity decreased in all patients.(ABSTRACT TRUNCATED AT 250 WORDS)     

The effects of verapamil on left ventricular function in the presence of beta-adrenergic blockade

Supraventricular tachyarrhythmias frequently complicate myocardial revascularization. Intravenous administration of verapamil has been effective in terminating these arrhythmias. To determine the effects of verapamil on left ventricular systolic function, we implanted ultrasonic dimension transducers in dogs and, after they had recovered from the operation, studied them while they were awake and unsedated. Intravenous administration of verapamil (0.2 mg/kg) resulted in an elevation of cardiac output above baseline because of reflex-induced tachycardia. Contractility, as measured by the load-independent end-systolic pressure-volume relationship, remained unchanged. When the animals were pretreated with atropine and propranolol, verapamil resulted in a fall in cardiac output and contractility. The intact animal responded to the vasodilatory effect of verapamil by releasing catecholamines to maintain cardiac output and hemodynamic stability. Only when this compensatory mechanism was blocked by a beta-adrenergic blocker do the inherently negative inotropic and chronotropic effects of verapamil become apparent. The clinical ramifications of this finding are of greater importance to the surgeon as more patients receive beta-adrenergic blocking agents up to the time of the immediate preoperative period. We conclude that verapamil should be administered with caution to patients with supraventricular tachyarrhythmias who have been receiving beta-adrenergic blocking agents.     

Hypothalamic-pituitary-gonadal function in two infants with Smith-Lemli-Opitz syndrome

We report on the hypothalamic-pituitary-gonadal function in 2 male infants with the Smith-Lemli-Opitz (SLO or RSH) syndrome. Both infants had abnormal external genitalia. Basal and LHRH stimulated plasma gonadotropins were normal for age (1 month). Plasma testosterone, androstenedione, and dehydroepiandrosterone sulfate were normal for age and sex. Some forms of congenital adrenal hyperplasia (17,20-desmolase deficiency, 17α-hydroxylase deficiency, and 3β-hydroxysteroid dehydrogenase deficiency) were ruled out by hormonal studies. The endocrinological findings indicate a normal hypothalamic-pituitary-gonadal function and a normal adrenal steroid biosynthesis in these 2 patients. A partial androgen receptor defect causing the genital malformations seems possible in one patient. Whether 5α-reductase deficiency is the cause of the male pseudohermaphroditism in SLO syndrome remains the subject of future studies. © 1992 Wiley-Liss, Inc.     

Considerations for the Use of Cytokine-Secreting Tumor Cell Preparations for Cancer Treatment

Limited efficacy of chemotherapy in most solid tumors has revived interest in immunotherapeutic approaches for cancer. One novel form of immunotherapy is the use of cancer vaccines consisting of tumor cells genetically engineered to secrete cytokines. The rationale for this immunization strategy is based on the existence of tumor-specific antigens, on the importance of the cellular arm of the immune system in mediating an effective antitumor response, and on the role of cytokines in regulating the cellular immune response. Such tumor vaccines showed considerable promise in various animal models and induced potent antitumor immunity in the host, which led to regression of established tumors and, moreover, produced immunological memory protecting animals from a subsequent tumor challenge at a distant site. Translated to the human patient, this implies that genetically modified tumor vaccines may be able to eradicate or reduce existing tumor deposits to subclinical levels as well as provide long-term protection from regrowth of tumor cells. This report will review and discuss the concept and rationale for the use of cytokine-secreting tumor vaccines for the treatment of human malignancies.     

Insulin-like growth-factor-binding protein 3 is decreased in early-stage operable pre-menopausal breast cancer

Insulin-like growth factor 1 (IGF-1) is a potent mitogen for human breast-cancer cells in vitro. In circulation, most of IGF-1 is bound to IGF-binding protein 3 (IGFBP-3). This high-affinity binding is thought to have an important limiting effect on the availability of IGF-1 for biological activity. To assess the availability of IGF-1 for receptor binding, we determined serum levels of IGF-1 and IGFBP-3 and IGF-1/IGFBP-3 ratios. In a case-control study, 150 women aged 38 to 75 years presenting with stage-l or-II breast cancer were investigated just prior to surgery (n = 76), or to irradiation one month after surgery (n = 74). The population-based control group consisted of 441 women of the same age having no breast cancer. Women reporting diabetes mellitus or other hormonal abnormalities were excluded. Premenopausal cases showed elevated IGF-1 serum concentrations, decreased IGFBP-3 levels and increased IGF-1/IGFBP-3 ratios. The IGF-1/IGFBP-3 ratio was a significant breast-cancer risk factor, also after adjustment for age, family history, height, body-mass index, body-fat distribution, and serum levels of C-peptide. The relative risk was 7.34 for the highest compared with the lowest quintile of IGF-1/IGFBP-3. The presence or absence of tumor had no influence on these results. Increased levels of available IGF-1 in the circulation of pre-menopausal women may contribute to the development of breast cancer. © 1995 Wiley-Liss Inc.     

NHS Direct usage in a GP population of children under 5 years: is NHS Direct used by people with the greatest health need?

This study compares health status, demographic and socioeconomic characteristics of users and non-users of NHS Direct in order to establish whether the service is being used by people with the greatest need for healthcare services. It suggests that use of NHS Direct is widespread among carers of children under 5 years old, but there is evidence that it may not be accessed equitably by those from ethnic minorities, lower socioeconomic groups and those with established ill health.     

Development of a short version of the Apathy Evaluation Scale specifically adapted for demented nursing home residents.

OBJECTIVE: Apathy is among the most frequent neuropsychiatric symptoms in dementia, particularly Alzheimer disease. The Apathy Evaluation Scale (AES) has been widely employed for assessing apathy in different patient groups. To further facilitate the usage of the AES, an abbreviated version was constructed. METHOD: On basis of a sample of 356 nursing home residents, a cross-validation procedure was carried out to develop a brief version of the AES. According to a thorough clinical examination, 85% of the residents were demented, 8% presented with mild cognitive impairment, whereas 7% did not present any cognitive deficits. After subdividing the patient group into two matched samples, the first subsample was used to identify problematic items due to defined psychometric and content-related criteria. The original 18-item scale was thus reduced to 10 items. Psychometric properties of the shortened version were subsequently reassessed in the second subsample. RESULTS: The short version demonstrated favorable psychometric properties that could be confirmed by cross-validation with the second sample. Correlations with the original full-length version were high (r = 0.97 for both subsamples); the shortened scale yielded no substantial losses regarding internal consistency or construct validity (correlations with the respective subscales of the Neuropsychiatric Inventory). CONCLUSION: The frequency of apathetic symptoms in the nursing home residents included confirms the clinical importance of apathy for understanding dementia. Given this specific patient population, setting, and mode of data collection, the short-version AES seems to be a valuable and time-efficient instrument for assessing apathy.