Long-term outcome in Susac syndrome

Susac syndrome is characterized by the clinical triad of encephalopathy, hearing loss, and retinal artery branch occlusions, mostly in young women. To our knowledge, long-term outcome and impact of pregnancy have not been specifically addressed. We report a series of 9 patients (7 female, 2 male) followed at the same institution, with special emphasis on clinical outcome including pregnancy and long-term sequelae. Clinical, brain magnetic resonance imaging (MRI), funduscopy, retinal angiography, and audiogram data were recorded every 3-12 months. We also analyzed the 92 previously reported cases of Susac syndrome. Mean follow-up was 6.4 years. Age at onset was 30.4 years. The first symptom occurred between April and September in 7 of 9 patients in the current study, and in 68% of all patients. The complete triad at onset was clinically obvious in only 1 of 9 patients. Brain involvement was heralded by headache and symptoms of encephalopathy. Cerebrospinal fluid was abnormal in 5 patients showing pleocytosis (mean, 24.6; range, 6-85 cells/mL) and elevated protein level (mean, 210; range, 113-365 mg/dL). Over time, quantitative brain MRI analysis showed that the number of lesions diminished and did not parallel clinical flares, and MRI never normalized. At the end of follow-up, no patient had severe impairment, and all but 1 returned to work. Inner ear involvement was present at onset in 2 patients and occurred in others with a mean delay of 11 months. Initially unilateral in 3, it became bilateral in all. Mean hearing loss was 34 dB (range, 15-70 dB). Hearing loss never improved, either spontaneously or under treatment. The eye was involved at onset in 8 patients, and after 3 years in 1. All had multiple bilateral retinal artery branch occlusions and/or dye leakage with hyperfluorescence of the arterial wall on fluorescein angiography. Over time, angiography normalized in 3 patients. In others, it was still abnormal at the end of follow-up (range, 1.5-10 yr). On late findings, fluorescein leakage was more frequent than true arterial occlusion. Eye involvement was mostly asymptomatic, unilateral, peripheral, and resumed spontaneously to remit in other sites over time. Corticosteroids were efficient to treat encephalopathy, with relapses occurring when the dosage was tapered. Steroid treatment did not improve hearing loss or prevent new retinal arteriolar occlusions. Anticoagulation had a role in treating encephalopathy and retinal arteriolar occlusions. Three patients had 4 pregnancies. Two pregnancies needed induced abortion. One pregnancy was uneventful. One pregnancy was complicated with Susac disease flare in the early postpartum period. In conclusion, at the end of follow-up, most patients had returned to work and none had severe impairment. Pregnancy may affect the course of Susac syndrome, with relapse of encephalopathy postpartum. Our main finding was that the course of Susac syndrome is not self-limited as previously thought, since isolated retinal arteriolar involvement may occur as a very late manifestation.     

The effects of fructo-oligosaccharides in combination with soy protein on bone in osteopenic ovariectomized rats

OBJECTIVE: The intestinal microflora is important in rendering soy isoflavones bioavailable by facilitating their conversion to equol. Hence, substances that can modulate the intestinal microflora could affect the bioavailability of isoflavones. In this study, we examined the effects of fructo-oligosaccharides (FOS), a prebiotic, on enhancing the effects of soy isoflavones on bone in ovariectomized osteopenic female rats. DESIGN: Sixty-three 9-month-old female Sprague-Dawley rats were either sham-operated (Sham; one group) or ovariectomized (Ovx; four groups) and were fed a control diet for 3 months to induce bone loss. After bone loss was confirmed via dual-energy x-ray absorptiometry, rats were placed on dietary treatment for 4 months. The Sham and one Ovx group received a control diet, and the remaining Ovx groups received either a soy protein-based diet (Soy), a FOS-supplemented diet (FOS), or a soy protein-based and FOS-supplemented diet (Soy+FOS). Before the termination of the study, whole-body bone mineral density (BMD) and bone mineral content (BMC) were assessed under anesthesia. Immediately after euthanasia, bone specimens were collected for the assessments of BMD, BMC, and biomechanical and microarchitectural properties. RESULTS: Whole-body BMD values were significantly higher in FOS and Soy+FOS groups compared with Ovx controls. The tibial BMC increased by 10%, 6%, and 4% in Soy, FOS, and Soy+FOS groups, respectively, compared to the Ovx control group. FOS and FOS+Soy treatments had the most pronounced effects in enhancing lumbar BMC and BMD. The FOS+Soy combination effectively improved tibial microarchitectural properties by enhancing trabecular number and lowering trabecular separation compared with Ovx controls. The effects of dietary treatments on lumbar microarchitectural properties were minimal and biomechanical properties of the femur were not affected by any of the dietary treatments. CONCLUSION: Our findings suggest that, although incorporation of either soy or FOS in the diet of Ovx rats can improve BMD of the whole body, tibiae, and lumbar vertebrae, their combination had no any additive effects. However, in terms of microarchitecture, the combination of soy and FOS had a greater effect in reversing the loss of certain microarchitectural parameters such as tibial trabecular number, separation, and thickness.     

The dilution of benzalkonium chloride (BAK) in the tear Film

The claim that benzalkonium chloride (BAK) synergistically enhances the antibiotic efficacy of gatifloxacin ophthalmic solution 0.3% (preserved with 0.005% BAK [50 μg/mL), Zymar; Allergan Inc., Irvine, Calif, USA) has been the subject of several studies. The purpose of this prospective clinical study was to test the hypothesis that BAK would be significantly diluted shortly after topical ocular administration and would thereafter have little or no effect on the enhancement of the antibiotic efficacy of commercial gatifloxacin on the ocular surface. This hypothesis was tested by investigators who measured the concentration of tear film BAK at successive time points after topical administration of commercial gatifloxacin. After subjects (N=10) received 5 separate instillations of a single 35-μL drop of gatifloxacin 0.3% ophthalmic solution in each eye, tear samples were collected at 30 sec, 1 min, 3 min, 5 min, and 20 min, with the use of graduated 5-μL glass microcapillaries. A validated high-performance liquid chromatography method was used to measure the concentration of BAK in each tear sample. The results showed rapid BAK dilution to 6.4 μg/mL, 3.2 μg/mL, 1.4 μg/mL, below the detection limit, and below the detection limit at 30 sec, 1 min, 3 min, 5 min, and 20 min after instillation of a single 35-μL drop of gatifloxacin. Because such rapid dilution reduces the concentration of BAK to near zero in minutes and does not allow the time (1 h) required for effective bacterial kill power, BAK is not expected to have a clinically significant effect on enhancement of the antimicrobial efficacy of gatifloxacin on the human ocular surface.     

Association of major dietary patterns with socioeconomic and lifestyle factors of adult women living in Tehran,Iran

ObjectivesTo detect major dietary patterns and their relation with demographic, socioeconomic, and lifestyle factors in adult women living in Tehran, Iran.MethodsIn a cross-sectional study, 460 women 20–50 y of age were selected by a stratified random-sampling method. Factor analysis was conducted to ascertain the major dietary patterns, and analysis of covariance was fitted to assess the relation between demographic, socioeconomic, and lifestyle variables and adherence to major dietary patterns.ResultsTwo major dietary patterns were extracted: healthy and unhealthy. After adjustment for confounders, age (b = 0.31, P < 0.01), university degree (b = 0.85, P < 0.01), housing size >20 m²/head (b = 0.30, P < 0.01), total family income US >$500/mo (b = 0.25, P < 0.05), physical activity (b = 0.01, P < 0.05), and history of hypertension (b = 0.36, P < 0.05) were positively associated and duration of residence in Tehran (b = ?0.01, P < 0.05) was negatively associated with a healthy dietary pattern. Conversely, ethnicity (Turk versus Fars; b = 0.30, P < 0.01) and smoking (b = 0.64, P < 0.01) were positively associated and age (b = ?0.33, P < 0.01) was negatively associated with an unhealthy dietary pattern.ConclusionOur findings support the association between demographic, socioeconomic, lifestyle factors and dietary patterns of the studied women. Therefore, it is suggested these factors should be considered in future studies on the association between dietary patterns and disease.     

Effects of silymarin on the proliferation and glutathione levels of peripheral blood mononuclear cells from beta-thalassemia major patients

Iron toxicity in beta-thalassemia major is the main cause of oxidative stress and cell mediated immune deficiencies. Despite indicative signs of severe oxidative deficiencies associated with beta-thalassemia major, such as decreased level of plasma antioxidants and depletion of erythrocyte glutathione, little is known about intracellular redox status of immune cells. Since glutathione is a primary intracellular antioxidant and plays an essential role in several functions in T cells, in this study intracellular glutathione (GSH) levels as well as proliferation of PHA-activated peripheral blood mononuclear cells (PBMC) were investigated in 28 beta-thalassemia major patients and 28 healthy age-matched individuals. Considering the potential benefits of flavonoids in the therapy of oxidative stress, the effects of silymarin on the GSH levels and proliferation of PBMC from normal and thalassemia individuals were further examined. Quantitative determination of intracellular GSH and proliferative response of PBMC to PHA were performed before and after 72 h incubation of PBMC with various concentrations of silymarin (0, 5, 10, or 20 mug/ml). Results demonstrated a significant reduction of GSH and proliferation in beta-thalassemia major cells; however treatment with silymarin led to restoration of both GSH levels and PBMC proliferation in thalassemia patients. Considerably low levels of GSH and depressed proliferative response of PBMC in beta-thalassemia major may be responsible for the cell mediated immune abnormalities in iron overload conditions. Moreover, the GSH restoration and improvement of PBMC growth by silymarin is a possible explanation for its recently reported antioxidant and immunostimulatory activities. These data suggest the benefit of using flavonoids to normalize immune dysfunction in beta-thalassemia major. The immunomodulatory effects of silymarin in beta-thalassemia major are currently under further investigation in a double blind clinical trial.     

Recurrent pneumothorax at an infant with miliary tuberculosis

A seven-month-old girl with miliary tuberculosis (Tbc) admitted to hospital due to development of acute dyspnoea and cyanosis at the end of third month of anti-Tbc therapy. Pneumothorax was evident at right lung with the chest radiography. A chest tube placed under water seal was applied. The patient healed up and then discharged. One week later, the patient admitted to hospital again, with same complaints due to pneumothorax at the same hemithorax. Same treatment was applied to the patient. Anti-Tbc therapy was stopped at the end of 12th month. Although, pneumothorax is a rare life-threatening complication of miliary Tbc, it's not seen only on admission or soon after beginning of the therapy, but it can be seen several months later during treatment. We want to report this case. That is the first case in which pneumothorax developed during therapy of an infant with miliary Tbc.