Concurrent multiple sclerosis and amyotrophic lateral sclerosis: where inflammation and neurodegeneration meet?

The concurrence of multiple sclerosis (MS) and amyotrophic lateral sclerosis (ALS) is exceedingly rare and the pathological features have not been examined extensively. Here we describe the key pathological features of a 40 year old man with pathologically confirmed concurrent MS and ALS

Facilitation of information literacy through a multilingual MOOC considering cultural aspects

Purpose The purpose of this paper is to demonstrate the rationale, technical framework, content creation workflow and evaluation for a multilingual massive open online course (MOOC) to facilitate information literacy (IL) considering cultural aspects. Design/methodology/approach A good practice analysis built the basis for the technical and content framework. The evaluation approach consisted of three phases: first, the students were asked to fill out a short self-assessment questionnaire and a shortened adapted version of a standardized IL test. Second, they completed the full version of the IL MOOC. Third, they were asked to fill out the full version of a standardized IL test and a user experience questionnaire. Findings The results show that first the designed workflow was suitable in practice and led to the implementation of a full-grown MOOC. Second, the implementation itself provides implications for future projects developing multilingual educational resources. Third, the evaluation results show that participants achieved significantly higher results in a standardized IL test after attending the MOOC as mandatory coursework. Variations between the different student groups in the participating countries were observed. Fourth, self-motivation to complete the MOOC showed to be a challenge for students asked to attend the MOOC as nonmandatory out-of-classroom task. It seems that multilingual facilitation alone is not sufficient to increase active MOOC participation. Originality/value This paper presents an innovative approach of developing multilingual IL teaching resources and is one of the first works to evaluate the impact of an IL MOOC on learners' experience and learning outcomes in an international evaluation study

PTEN regulates AMPA receptor-mediated cell viability in iPS-derived motor neurons

Excitatory transmission in the brain is commonly mediated by the α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptors. In amyotrophic lateral sclerosis (ALS), AMPA receptors allow cytotoxic levels of calcium into neurons, contributing to motor neuron injury. We have previously shown that oculomotor neurons resistant to the disease process in ALS show reduced AMPA-mediated inward calcium currents compared with vulnerable spinal motor neurons. We have also shown that PTEN (phosphatase and tensin homolog deleted on chromosome 10) knockdown via siRNA promotes motor neuron survival in models of spinal muscular atrophy (SMA) and ALS. It has been reported that inhibition of PTEN attenuates the death of hippocampal neurons post injury by decreasing the effective translocation of the GluR2 subunit into the membrane. In addition, leptin can regulate AMPA receptor trafficking via PTEN inhibition. Thus, we speculate that manipulation of AMPA receptors by PTEN may represent a potential therapeutic strategy for neuroprotective intervention in ALS and other neurodegenerative disorders. To this end, the first step is to establish a fibroblast–iPS–motor neuron in vitro cell model to study AMPA receptor manipulation. Here we report that iPS-derived motor neurons from human fibroblasts express AMPA receptors. PTEN depletion decreases AMPA receptor expression and AMPA-mediated whole-cell currents, resulting in inhibition of AMPA-induced neuronal death in primary cultured and iPS-derived motor neurons. Taken together, our results imply that PTEN depletion may protect motor neurons by inhibition of excitatory transmission that represents a therapeutic strategy of potential benefit for the amelioration of excitotoxicity in ALS and other neurodegenerative disorders