Most types of electrographic epileptiform activity can be characterized by isolated or repetitive bursts in brain electrical activity. This observation is our motivation to determine mechanisms that underlie bursting behavior of neuronal networks. Here we show that the persistent sodium current (Na P) in mouse neocortical slices is associated with cellular bursting, and our data suggests that t...

Background: Opioid sensitivity is well known to vary widely among individual subject in levels of μ-opioid receptor (MOP) expression, responses to pain and analgesic effect of morphine. Many studies have reported that the single nucleotide polymorphism (SNP), A118G, of the μ-opioid receptor gene (OPRM1) has been shown to be associated with MOP expression levels, as well as pain thresholds, anal...

BACKGROUND Riluzole is the only drug to prolong survival for amyotrophic lateral sclerosis (ALS) and, at a dose of 100 mg, was associated with a 35% reduction in mortality in a clinical trial. A key question is whether the survival benefit occurs at an early stage of disease, late stage, or is spread throughout the course of the disease. To address this question, we used the King's clinical sta...

Huntington's disease (HD) is an autosomal dominant, inherited and fatal neurodegenerative disorder for which there is, at present, no effective treatment or cure. Striatal medium spiny neurons (MSN) are the most sensitive in HD. Dysregulation of glutamate/calcium signaling pathway emerges as a possible cause of striatal MSN neurodegeneration in HD. Here we evaluated five clinically relevant glu...

Decrement of the thenar compound muscle action potentials (CMAP), after repetitive nerve stimulation (RNS) of the median nerve at 3 Hz, was evaluated in patients with ALS before riluzole therapy. CMAP size as well as motor unit number and size estimates were evaluated twice before and after 1 year of riluzole therapy. The correlation between decrement and CMAP size reduction per year was highly...

Parkinson plus diseases, comprising mainly progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) are rare neurodegenerative conditions. We designed a double-blind randomized placebo-controlled trial of riluzole as a potential disease-modifying agent in Parkinson plus disorders (NNIPPS: Neuroprotection and Natural History in Parkinson Plus Syndromes). We analysed the accuracy of...

decremental responses to repetitive nerve stimulation in ALS patients Article abstract—Decrement of the thenar compound muscle action potentials (CMAP), after repetitive nerve stimulation (RNS) of the median nerve at 3 Hz, was evaluated in patients with ALS before riluzole therapy. CMAP size as well as motor unit number and size estimates were evaluated twice before and after 1 year of riluzole...