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Professor Winder's group at the University of Sheffield investigating the cancer drug, dasatinib, a potent and specific Src tyrosine kinase inhibitor has shown that it decreases the levels of β-dystroglycan phosphorylation on tyrosine and to increase the relative levels of non-phosphorylated β-dystroglycan in dystrophic sapje zebrafish. Tyrosine phosphorylation and degradation of β-dystroglycan is a key event in the aetiology of Duchenne muscular dystrophy. Dasatinib treatment resulted in the improved physical appearance of the sapje zebrafish musculature and increased swimming ability as measured by both duration and distance of swimming of dasatinib-treated fish compared with control...

Researchers have shown that they were able to improve muscle function in  Duchenne Muscular Dystrophy mice using in vivo gene editing techniques. Duchenne muscular dystrophy (DMD) affects about 1 out of 5000 male births and caused by mutations in the dystrophin gene. Though DMD has been a target for gene therapy for a long time, progress has been very slow and attempts unsuccessful.The dystrophin gene has 79 sections, or exons, but can retain reasonable function even if a few exons in the middle are lost. Dystrophin works as long as its two ends are intact...

In a paper published on Jan. 4, 2016, in the online Early Edition of the journal Proceedings of the National Academy of Sciences, researchers from Penn State University reports on the discovery of a novel drug target, which could help in the treatment for Rett Syndrome and other forms of autism-spectrum disorders. In this work, the researchers demonstrate that human neurons derived from induced pluripotent stem cells (iPSCs) from patients with Rett syndrome (Rett neurons), show a significant deficit in neuron-specific K+-Cl− cotransporter2 (KCC2) expression, leading to an impaired GABA functional switch from excitation to...

Charles Kennedy, one of the first child neurologists in the USA, passed away on October 6, 2015 in Maine at the age of 95 following a brief illness. Born in Buffalo, NY, he attended Nichols School and later Deerfield Academy. He graduated from Princeton University with honors in Chemistry in 1942. At Princeton, in addition to preparing for medical school, he was also a member of the choir, glee club and the Princeton Nassoons (an all male a cappella group). He went on to receive his MD degree at the University of Rochester...

The 2nd meeting of the Posterior Fossa Society will take place at the Convention Centre Liverpool on Sunday, June 12th 2016. The Posterior Fossa Society is an international group of researchers and health care professionals (doctors, nurses, psychologists, speech pathologists, linguists, neuroscientists etc.) who are dedicated to research into the causes, features, treatment and prevention of the postoperative Cerebellar Mutism Syndrome (CMS) and Cerebellar Cognitive Affective Syndrome (CCAS) in children and adults. It reaches its goals by systematically gathering and exchanging information on the CMS and CCAS, both online and during international meetings and conferences....

Twelfth Göttingen Meeting of the German Neuroscience Society (March 22 – 25, 2017) The Göttingen Meeting of the German Neuroscience Societies is one of the largest multi-disciplinary neuroscience meetings in Europe with around 1.700 participants. It covers a wide range of research fields in the neurosciences including vertebrate and invertebrate systems, molecular, cellular and systemic neurobiology, neuropharmacology, developmental, computational, behavioral, cognitive and clinical neuroscience. The call for symposia for the 12th Göttingen Meeting of the German Neuroscience Society is open now. Symposia dealing with all areas of neuroscience research are invited. The...

There have been nearly 60 cases identified in California from 2012 - 2015 of acute flaccid myelitis, a rare syndrome described as polio-like, with most patients being children and young adults, according to a study in the December 22/29 issue of JAMA. The cause of the condition in these cases remains unknown. With the elimination of wild poliovirus in populations throughout most of the world, the clinical syndrome of acute flaccid paralysis (characterized by weakness or paralysis and reduced muscle tone) due to spinal motor neuron injury has largely disappeared from North America. Despite occasional...

Researchers have discovered how immune cells triggered by recurrent Strep A infections enter the brain, causing inflammation that may lead to autoimmune neuropsychiatric disorders in children. The study, performed in mice, found that immune cells reach the brain by traveling along odor-sensing neurons that emerge from the nasal cavity, not by breaching the blood-brain barrier directly. The findings could lead to improved methods for diagnosing, monitoring, and treating these disorders. The study, led by researchers at Columbia University Medical Center (CUMC) and the University of Minnesota, Minneapolis, was published today in the online edition of...

Studies in rats have shown that they could be either woken up or put in an unconscious state from altering their brain activity by changing the firing rates of neurons in the central thalamus. The NIH funded study was published in eLIFE. Located deep inside the brain the thalamus acts as a relay station sending neural signals from the body to the cortex. Damage to neurons in the central part of the thalamus may lead to problems with sleep, attention, and memory. Previous studies have suggested that stimulation of thalamic neurons may...

Results from the ICISS trial presented at the 69th Annual Meeting of the American Epilepsy Society (AES) suggest that a combination of hormonal therapy with vigabatrin reduces infantile spasms better than hormonal treatment alone. For this study, the ICISS trial researchers tested the hypothesis that combining prednisolone or tetracosactide with vigabatrin would result in a greater proportion of infants achieving spasm cessation compared with hormonal therapy alone. Between March 2007 and May 2014, infants with IS and a compatible EEG were enrolled in a multicenter treatment trial. Infants were randomized to receive either hormonal therapy...