ABSTRACT
Mild encephalopathy/encephalitis with a reversible spleniat (MERS) lesion is a clinic-radiological entity.The clinical features of MERS in neonates are still not systemically reported.This paper presents five cases of MERS,and the up-to-date reviews of previously reported cases were collected and analyzed in the literature.Here we describe five cases clinically diagnosed with MERS.All of them were neonates and the average age was about 4 days.They were admitted for the common neurological symptoms such as hyperspasmia,poor reactivity and delirium.Auxiliary examinations during hospitalization also exhibited features in common.In this report,we reached following conclusions.Firstly,magnetic resonance imaging revealed solitary or comprehensive lesions in the splenium of corpus callosum,some of them extending to almost the whole corpus callosum.The lesions showed low intensity signal on Tl-weighted images,homogeneously hyperintense signal on T2-weighted images,fluid-attenuated inversion recovery and diffusion-weighted images,and exhibited an obvious reduced diffusion on apparent diffusion coefficient map.Moreover,the lesions in the magnetic resonance imaging disappeared very quickly even prior to the clinical recovery.Secondly,all the cases depicted here suffered electrolyte disturbances especially hyponatremia which could be easily corrected.Lastly,all of the cases recovered quickly over one week to one month and majority of them exhibited signs of infections and normal electroencephalography.
ABSTRACT
The effects of Sonic hedgehog (Shh) signaling pathway activation on S-type neuroblastoma (NB) cell lines and its role in NB tumorigenesis were investigated. Immunohistochemistry was used to detect the expression of Shh pathway components-- Patchedl (PTCH1) and Glil in 40 human primary NB samples. Western blotting and RT-PCR were used to examine the protein expression and mRNA levels of PTCH1 and Glil in three kinds of S-type NB cell lines (SK-N-AS, SK-N-SH and SHEPI), re-spectively. Exogenous Shh was administrated to activate Shh signaling pathway while cyclopamine was used as a selective antagonist of Shh pathway. S-type NB cell lines were treated with different concen-trations of Shh or/and cyclopamine for different durations. Cell viability was measured by using MTT method. Apoptosis rate and cell cycle were assayed by flow cytometry. The xenograft experiments were used to evaluate the role of Shh pathway in tumor growth in immunodeficient mice. High-level expres-sion of PTCH1 and Gill was detected in both NB samples and S-type NB cell lines. Cyclopamine de-creased the survival rate of the three cell lines while Shh increased it, and the inhibition effects of cyclopaminc could be partially reversed by shh pre-treatment. Cyclopamine induced the cell apoptosis and the cell cycle arrest in G0/G1 phase, while Shh induced the reverse effects and could partially pre-vent effects of cyclopamine. Cyclopamine could also inhibit the growth of NB in vivo. Our studies re-vealed that activation of the Shh pathway is important for survival and proliferation of S-type NB cells in vivo and in vitro through affecting cell apoptosis and cell cycle, suggesting a new therapeutic ap-proach to NB.