A Young Woman With Hypertonia, Severe Scoliosis, and Encephalopathy.

A 31-year-old woman was seen with contractures in her fingers and toes, carpal inversion, dysarthria, dysphagia, hypertonia, decreased tendon reflexes, absence of Babinski sign, and no psychiatric problems and significant global atrophy. What is your diagnosis?

Inflammatory cytokines expression in Wilson's disease.

BACKGROUND: Wilson's disease (WD) is an autosomal recessive inherited disorder of copper (Cu) metabolism. Inflammation is a self-defensive reaction aimed at eliminating or neutralizing injurious stimuli, and restoring tissue integrity. Copper deposition may lead to inflammation in the organs and tissues of WD patients. OBJECTIVE: The aim of this study was to compare the plasma levels of inflammatory cytokines in patients with WD and healthy group, and also to assess whether inflammatory cytokines affects the clinical manifestation of WD. METHODS: Ninety-nine patients with WD and 32 controls were recruited for this study. Ray Biotech antibody microarray was used to detect the levels of plasma inflammatory cytokines. RESULTS AND CONCLUSION: Our results showed significant increase in T helper (Th) 1 cells (IL-2, TNF-α, and TNF-ß), Th2 cells (IL-5, IL-10, and IL-13), and Th17 (IL-23) (p < 0.05). Higher plasma Th 1 cells (IL-2, TNF-α, and TNF-ß), Th 2 cells (IL-13), and Th 17 (TGF-ß1, IL-23) levels were found in neurological patients compared with control groups (p < 0.01). Besides, we found Th 1 cells (TNF-α and TNF-ß), Th 3 (TGF-ß1), and Th 17 (IL-23) levels were significantly higher in hepatic and neurological patients (p < 0.05). In addition, the higher Th1 cells (IL-2, TNF-α, and TNF-ß), Th2 cells (IL-13), and Th17 (TGF-ß1, IL-23) and the course of WD were associated with the severity of the neurological symptoms for WD patients. Altogether, our results indicated that dysregulation of cytokines, mainly increased expression of cytokines and chemokines, occurred in WD patients.

An unusual alkylidyne homologation.

The reaction of [W([triple bond, length as m-dash]CH)Br(CO)2(dcpe)] (dcpe = 1,2-bis(dicyclohexylphosphino)ethane) with tBuLi and SiCl4 affords the trichlorosilyl ligated neopentylidyne complex [W([triple bond, length as m-dash]CtBu)(SiCl3)(CO)2(dcpe)]. This slowly reacts with H2O to afford [W([triple bond, length as m-dash]CCH2tBu)Cl3(dcpe)] and ultimately H2C[double bond, length as m-dash]CHtBu via an unprecedented alkylidyne homologation in which coordinated CO is the source of the additional carbon atom with potential relevance to the Fischer-Tropsch process.

Rearrangement of bis(alkylidynyl)phosphines to phospha-acyls.

A range of bis(alkylidynyl)phosphines RP{C[triple bond, length as m-dash]M(CO)2(Tp*)}2 (M = Mo, W; R = Cl, Ph, Cy; Tp* = hydrotris(dimethylpyrazolyl)borate) are obtained from the reactions of [M([triple bond, length as m-dash]CLi)(CO)2(Tp*)] with Cl2PR or alternatively via the palladium(0)-mediated reactions of [W([triple bond, length as m-dash]CBr)(CO)2(Tp*)] with RPH2 (R = Py, Cy). The complexes RP{C[triple bond, length as m-dash]W(CO)2(Tp*)}2 rearrange slowly (R = Cl) or on heating (R = Ph) to afford the isomeric phospha-acyls [W2(µ:η1-C;η2-C,P-CCPR)(CO)4(Tp*)2].