Corticomotoneuronal dysfunction in ALS patients with different SOD1 mutations.

OBJECTIVE: To examine corticomotoneuronal function in amyotrophic lateral sclerosis (ALS) patients carrying superoxide dismutase 1 (SOD1) mutations using peristimulus time histograms (PSTH). METHODS: Six I113T, 3 A4V, one G41D and one G114A patient were studied along with 21 healthy control subjects. Analyses included comparison with previously reported data from 8 D90A homozygous and 12 sporadic ALS (SALS) patients examined by the authors using identical methodology. RESULTS: Cortical threshold was significantly reduced in A4V patients (41.3%) compared to I113T (58%), SALS (57%) and D90A (71%) patients, as well as healthy controls (49.7%). Estimated excitatory postsynaptic potentials (EPSPs) were significantly larger in A4V patients (4.39 mV) compared to healthy controls (2.95 mV), I113T (2.71 mV) and SALS (2.39 mV) patients. Clinical features and PSTH parameters in I113T were similar to SALS, however, PSTH primary peaks (PP) were significantly more dispersed, 9.5 ms compared to 4ms in SALS. PSTHs from single G41D and G114A patients were unremarkable, apart from large EPSP amplitudes in the G114A patient. CONCLUSIONS: ALS patients with A4V and I113T SOD1 mutations have distinctive corticomotoneuronal changes that are different from those in D90A homozygous and SALS patients. SIGNIFICANCE: PSTH studies should be considered for future in vivo studies of SOD1 pathophysiology in ALS.     

Combining treatment for written and spoken naming.

Individuals with left-hemisphere damage often have concomitant impairment of spoken and written language. Whereas some treatment studies have shown that reading paired with spoken naming can benefit both language modalities, little systematic research has been directed toward the treatment of spelling combined with spoken naming. The purpose of this study was to examine the therapeutic effect of pairing a lexical spelling treatment referred to as Copy and Recall Treatment (CART) with verbal repetition of target words. This approach (CART + Repetition) was compared with treatment using verbal repetition without the inclusion of orthographic training (Repetition Only). Two individuals with moderate aphasia and severe impairment of spelling participated in the study using a multiple baseline design across stimulus sets and treatment conditions. Both participants improved spelling of targeted words as well as spoken naming of those items, but improvement in spoken naming was marked for one individual in the CART + Repetition condition, while the other participant made smaller gains in spoken than written naming irrespective of treatment condition. Consideration of the participant profiles suggested that CART + Repetition provides greater benefit when there is some residual phonological ability and the treatment serves to stimulate links between orthography and phonology.     

What should nurse teachers be doing? A preliminary study

There is little doubt that the role of the nurse teacher is complex and multifaceted in nature. The apparently conflicting demands of NHS trusts and education in the United Kingdom, at the time of integration of nurse education into the higher education sector, further complicates the role. This recognition underlines the need to capture and measure key aspects of nurse teacher function in order to maximize educational gain. Using a comprehensive literature review, focus group discussions and a small number of individual semi-structured interviews, this preliminary study explored the issue of teacher workload, from the perspective of the principal stakeholders in nurse and higher education. Competing research and clinical practice objectives for nurse teachers are identified against a background of changing health care provision, reduced availability of placements and an increased need for evidence-based practice.     

DNA-based vaccines: new possibilities for disease prevention and treatment.

Ottawa researcher Dr. Heather L. Davis has become a pioneer in the development of DNA-based vaccines. With her collaborators in France and Germany Davis found that introducing the DNA code for the envelope protein of the hepatitis B virus into the muscle tissue of mice prompted a strong and sustained immune response. She believes that DNA-based vaccines could prove to offer many advantages over conventional vaccines, not least of which are greater safety and effectiveness and reduced cost. She has also begun to explore the potential of DNA-based vaccines for use in the treatment of disease. In recent experiments Davis and colleagues in France have successfully used DNA vaccination to cure transgenic mice of a chronic hepatitis B carrier state.     

Successful Treatment of Kasabach-Merritt Syndrome with Prednisone and Epsilon-Aminocaproic Acid

The Kasabach-Merritt syndrome is characterized by thrombocytopenia and localized coagulopathy associated with a hemangioma. Most techniques applied to eradicate the tumor or accelerate its involution (surgery, radiation therapy, embolization) are invasive and require transfusion of large amounts of blood products. In some cases, medical treatment is the only alternative. Efficacy of steroids and antifibronolytic agents has already been described, but even this approach is associated with the administration of blood products. We report two cases of infants with Kasabach-Merritt syndrome associated with cardiac and hepatic hemangiomas. At admission, both had signs of cardiac failure. They were successfully treated with prednisone and epsilon-aminocaproic acid (EACA). Blood products were not required once the diagnosis was made. These observations have important implications for the management of patients with Kasabach-Merritt syndrome because they show that even in severe cases blood transfusions can be avoided by the use of prednisone and EACA.