Search

Myotonic dystrophy type 2 (DM2) is a multisystem disorder that affects many organs and systems, including the brain. The objective is to analyze personality patterns in myotonic dystrophy type 2 (DM2) compared to DM1 control group. The study comprised 27 consecutive genetically confirmed DM2 patients and control group of 44 DM1 patients. Personality traits were assessed with the Millon Multiaxial Clinical Inventory III (MMCI III). In DM2 group there were no scale with pathological scores, although compulsive and paranoid traits were the most prominent. DM2 patients had lower scores compared to DM1 patients in almost all scales. Pathological scores on clinical symptom scales were not observed, although anxiety scale almost approached this value. Patients with higher compulsive score had higher level of education (rho = +0.53, p < 0.01). On the other hand, higher paranoid score correlated with younger age at onset (rho = -0.34, p < 0.01) and lower educational level (rho = -0.26, p < 0.05). Our results did not show significant personality impairments in patients with DM2. However, following personality traits were predominant: compulsive (in patients with higher education) and paranoid (in patients with lower education and earlier age at onset). The most common clinical symptoms were anxiety and somatization.

Importance Low-molecular-weight heparin is recommended over warfarin for the treatment of acute venous thromboembolism (VTE) in patients with active cancer largely based on results of a single, large trial. Objective To study the efficacy and safety of tinzaparin vs warfarin for treatment of acute, symptomatic VTE in patients with active cancer. Design, Settings, and Participants A randomized, open-label study with blinded central adjudication of study outcomes enrolled patients in 164 centers in Asia, Africa, Europe, and North, Central, and South America between August 2010 and November 2013. Adult patients with active cancer (defined as histologic diagnosis of cancer and receiving anticancer therapy or diagnosed with, or received such therapy, within the previous 6 months) and objectively documented proximal deep vein thrombosis (DVT) or pulmonary embolism, with a life expectancy greater than 6 months and without contraindications for anticoagulation, were followed up for 180 days and for 30 days after the last study medication dose for collection of safety data. Interventions Tinzaparin (175 IU/kg) once daily for 6 months vs conventional therapy with tinzaparin (175 IU/kg) once daily for 5 to 10 days followed by warfarin at a dose adjusted to maintain the international normalized ratio within the therapeutic range (2.0-3.0) for 6 months. Main Outcomes and Measures Primary efficacy outcome was a composite of centrally adjudicated recurrent DVT, fatal or nonfatal pulmonary embolism, and incidental VTE. Safety outcomes included major bleeding, clinically relevant nonmajor bleeding, and overall mortality. Results Nine hundred patients were randomized and included in intention-to-treat efficacy and safety analyses. Recurrent VTE occurred in 31 of 449 patients treated with tinzaparin and 45 of 451 patients treated with warfarin (6-month cumulative incidence, 7.2% for tinzaparin vs 10.5% for warfarin; hazard ratio [HR], 0.65 [95% CI, 0.41-1.03]; P = .07). There were no differences in major bleeding (12 patients for tinzaparin vs 11 patients for warfarin; HR, 0.89 [95% CI, 0.40-1.99]; P = .77) or overall mortality (150 patients for tinzaparin vs 138 patients for warfarin; HR, 1.08 [95% CI, 0.85-1.36]; P = .54). A significant reduction in clinically relevant nonmajor bleeding was observed with tinzaparin (49 of 449 patients for tinzaparin vs 69 of 451 patients for warfarin; HR, 0.58 [95% CI, 0.40-0.84]; P = .004). Conclusions and Relevance Among patients with active cancer and acute symptomatic VTE, the use of full-dose tinzaparin (175 IU/kg) daily compared with warfarin for 6 months did not significantly reduce the composite measure of recurrent VTE and was not associated with reductions in overall mortality or major bleeding, but was associated with a lower rate of clinically relevant nonmajor bleeding. Further studies are needed to assess whether the efficacy outcomes would be different in patients at higher risk of recurrent VTE. Trial Registration clinicaltrials.gov Identifier:NCT01130025

Contains fulltext : 154072.pdf (Publisher’s version ) (Closed access)

A simple method for approximation of all-pole recursive digital filters, directly in digital domain, is described. Transfer function of these filters, referred to as Ultraspherical filters, is controlled by order of the Ultraspherical polynomial, nu. Parameter nu, restricted to be a nonnegative real number (nu ≥ 0), controls ripple peaks in the passband of the magnitude response and enables a trade-off between the passband loss and the group delay response of the resulting filter. Chebyshev filters of the first and of the second kind, and also Legendre and Butterworth filters are shown to be special cases of these allpole recursive digital filters. Closed form equations for the computation of the filter coefficients are provided. The design technique is illustrated with examples.

A disease consisting of persistent muscle cramps involving distal muscle groups that occurred in 12 members of the same family is described. The cramps appeared on exertion and in full relaxation or during sleep. In the third generation they appeared in the second decade; in the fourth and fifth generations in childhood with higher frequency and intensity of cramps. The disease is not sex linked and seems to be dominantly inherited. Electromyography showed no myotonic response on insertion. Motor unit potentials were normal. Continual waxing and waning electrical discharges corresponding to clinically visible contractions of parts of the muscles were present. Repetitive nerve stimulation caused no change in the amplitude of evoked muscle potentials. On spinal anaesthesia or nerve block the muscle contractions continued but became painless. The movements were only stopped with local infiltration of anaesthetic into the muscle. There were no cramps on ischaemic work. Drug studies revealed no benefit on carbamazepine, slight relief with meprobamate, and complete disappearance with potassium chloride. The remission outlasted the treatment for three months and then cramps of milder degree reappeared. Repeated potassium chloride treatment was not effective. The cramps increased on hydrochlorothiazide, and 12 hours after spinal anaesthesia. In the authors' opinion the disease should be considered as not belonging to any known nosological entity.

Communication orale. MED-ESCWET project; International audience