A fourth ventricular ganglioneurocytoma representing with cerebellar epilepsy: a case report and review of the literature.

Fourth ventricular low-grade tumoral or dysplastic neuronal lesions have been reported as an epileptic focus for recently described cerebellar epilepsy in the form of repetitive and stereotyped attacks of hemifascial spasm, eye blinking, fascial movements, head deviation and dysautonomic manifestations. The case of a 3-month old infant having fourth ventricular mass with similar symptoms such as paroxysmal facial movements, eye blinking, eyelid contractions and abnormal head posture is reported in this article. After a few days of her admission, her attacks displayed a new form with altered consciousness and left limb jerks which were unresponsive to medical therapy. Following the surgical excision of the lesion 10 months ago, attacks disappeared and she is still seizure free. Histopathological diagnosis was ganglioneurocytoma. The seizures (which may be intractable in cerebellar epilepsy) are thought to have arisen from subcortical structures such as cerebellum, brain stem nuclei or the lesion itself. In the case of intractable episodes, surgical excision may prevent further seizures and help patients have a normal cognitive and motor development.     

Fatal GvHD as a complication of liver transplantation for undetermined fulminant hepatic failure and associated aplastic anemia.

Fulminant hepatic failure of unknown origin is the most common cause of fulminant hepatitis with high incidence of aplastic anaemia. Furthermore, the association of liver failure and aplastic anaemia has an increased mortality rate. In this report we describe a 16-month-old boy who presented with aplastic anaemia preceding a non-A, non-B, non-C fulminant liver failure. He developed severe graft versus host disease (GvHD) after liver transplantation, proven by the presence of donor cells in the peripheral blood and in the skin biopsy. He received conventional therapy (steroids, mycophenolate, anti-IL-2 monoclonal antibodies, anti-thymocyte globulin) without success. In an attempt to obtain T cell depletion and reduce the GvHD, he was treated with Alemtuzumab, a first time use for this indication. Aplastic anaemia was extensively investigated, especially exploring the possibility of primary immunodeficiency and reticular dysgenesis which were excluded based on clinical history. However, another form of primary immunodeficiency could be the cause of the uncontrollable proliferation of the donor lymphocytes derived from the liver transplant. Despite aggressive treatment GvHD progressed and the patient died of multiorgan failure. The majority of authors mention aplastic anaemia as a secondary event post liver transplant, whereas in our view this might be a haematopoietic stem cell disorder preceding fulminant hepatic failure. These patients also need to be evaluated extensively in order to exclude a primary immunodeficiency. The underlying disease will determine the choice of immunosuppressive treatment, especially in case of development of GvHD caused by the transplanted lymphocytes inhabiting the donor liver.     

Improved survival of transplanted cortical brain tissue grafts by iloprost in rats.

The cytoprotective effect of iloprost on the viability and survival of embryonic cortical brain tissue grafts was examined ultrastructurally under light and electron microscopy before and 4 weeks after transplantation surgery. It was shown that neural grafts stored in iloprost solution (50 ng/mL) for 3 hours were more or less in a normal cytoarchitecture compared with saline-preserved grafts. Moreover, it was demonstrated that 4 weeks after transplantation, graft tissues stored in iloprost solution for 3 hours before implantation maintained a successful survival. Thus, a higher cellular population with new vascularization areas and preservation of myelin formation were accepted as a desirable integration of the graft tissue into the host brain tissue. The mechanism of action of iloprost is discussed.     

Investigating the protective effect of melatonin on liver injury related to myocardial ischemia-reperfusion.

BACKGROUND: An animal model of myocardial ischemia-reperfusion (MI/R) was used to test the hypothesis that free radicals released with MI/R have hazardous effects on liver through remote organ injury. MATERIAL/METHODS: Twenty-one rats were divided into three groups: sham-treated, MI/R, and MI/R+melatonin. To produce MI/R, a branch of the left coronary artery was occluded for 30 min followed by two hours of reperfusion. Melatonin or vehicle was given 10 min before ischemia. At the end of the study, liver tissue was obtained for biochemical determination. The false discovery rate (FDR) is explained and was used for multiple comparisons of the groups' means. RESULTS: Compared with the sham group, MI/R significantly decreased glutathione (GSH) content and increased levels of nitric oxide (NO) and malondialdehyde (MDA). Melatonin administration significantly increased GSH levels and decreased the levels of NO and MDA compared with the MI/R group. CONCLUSIONS: Melatonin could prevent liver damage due to its strong antioxidant and free radical scavenger effects. Therefore, melatonin may have beneficial effects on remote organ injury such as MI/R-induced liver disorders.     

Decrease in epidermal CD44 expression as a potential mechanism for abnormal hyaluronate accumulation in superficial dermis in lichen sclerosus et atrophicus

CD44 is a polymorphic integral membrane glycoprotein that serves as the principal cell surface receptor for hyaluronate, the major component of the extracellular matrix. CD44 is abundantly found in the skin and functions as a cell adhesion molecule. In a recent study we have observed a massive dermal accumulation of hyaluronate as a result of the in vivo selective suppression of CD44 in keratinocytes in mice expressing a keratin 5 promoter-driven CD44 anti-sense transgene. As the histologic features of the dorsal skin of these transgenic mice display some similarities to those of the skin lesions of lichen sclerosus et atrophicus, we explored the nature of the material accumulated in the dermis of genital and extragenital lesions of 14 patients with lichen sclerosus et atrophicus by Alcian Blue and human CD44 receptor globulin stainings, as well as the epidermal expression of CD44 protein and mRNA by immunohistochemistry and in situ hybridization. In this study we provide evidence that hyaluronate is accumulated in the superficial dermis of lichen sclerosus et atrophicus lesions, in particular by the use of human CD44 receptor globulin staining, which binds specifically to hyaluronate. In addition we show that the protein and mRNA expression of CD44 in the epidermis of the involved lichen sclerosus et atrophicus skin from genital and extragenital areas is significantly decreased, and in some cases completely lost. In contrast, keratinocyte CD44 expression was un-altered in the skin lesions of lupus erythematosus, scleroderma and reticular erythematous mucinosis, despite the presence of a mucinous material in the dermis. These results suggest that a decrease in CD44 in the keratinocytes may be correlated with an abnormal dermal accumulation of hyaluronate in the lesions of lichen sclerosus et atrophicus, and may play a pathogenetic role in this disease. J Invest Dermatol 115:1054-1058 2000     

Early oral hydration after cesarean delivery performed under regional anesthesia

OBJECTIVE: To establish an early, safe, and well-tolerated time to start oral hydration after cesarean delivery. METHODS: A prospective randomized trial of women delivered by cesarean under regional anesthesia at Alanya Research and Medical Center, Turkey, between 2005 and 2006. A clear liquid drink was given to 100 women in the early oral intake (EOI) group 2 hours after surgery, and to 100 women in the delayed oral intake (DOI) group 8 hours after surgery. If the drink was tolerated, solid foods were then given gradually. RESULT: In the EOI and DOI groups, respectively, the mean time for the return of bowel movements was 4.4+/-1.9 h vs 6.3+/-2.4 h; mean time elapsed until initial passage of flatus was 12.1+/-5.5 h vs 24.1+/-6.0 h; and mean duration of hospitalization was 29.6+/-5.5 h vs 44.6+/-6.8 h (P<0.05). CONCLUSION: Following cesarean delivery under regional anesthesia, oral hydration given as early as 2 hours after the operation is safe and well tolerated.