Neurology
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To determine the rate of disease progression in Charcot-Marie-Tooth disease type 1A (CMT1A). ⋯ Progression of CMT1A can be detected by both the CMT Neuropathy Score (CMTNS) and the Neuropathy Impairment Score (NIS). This supports the feasibility of clinical trials to detect a slowing of disease progression using either or both of these scales as outcome measures. Since the CMTNS combines symptoms, signs, and electrophysiology and the NIS is based solely on the neurologic examination, the two scales may be complementary.
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In amyotrophic lateral sclerosis (ALS), the origin of fasciculations is disputed. We hypothesized that the discharge pattern of fasciculation potentials (FPs) would be different for FPs arising in the motor axon or in the spinal motor neuron. ⋯ Firing pattern analysis, based on high-density surface EMG, can detect fasciculation potentials (FPs) of axonal and neuronal origin in amyotrophic lateral sclerosis. The two FP types coexist within the same muscle. The recognition that clinically identical fasciculations conceal the existence of two types of FP that can be studied in a noninvasive manner will introduce a new aspect in the research of motor neuron disease.
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Several recent randomized clinical trials have found that the medications being evaluated for neuropathic pain did not significantly differ from placebo for the primary efficacy endpoint, despite encouraging results from prior preclinical and clinical studies. It is unclear whether these trials were unsuccessful because the medications truly lack efficacy or whether characteristics of the trials compromised the demonstration of treatment benefits. ⋯ The results suggest that study characteristics may contribute to the outcomes of clinical trials of treatments for neuropathic pain and provide an impetus for investigating strategies for decreasing placebo response rates and thereby possibly increasing the likelihood of positive outcomes in trials of efficacious treatments.
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Comparative Study
Variability of brain death determination guidelines in leading US neurologic institutions.
In accordance with the Uniform Determination of Death Act, guidelines for brain death determination are developed at an institutional level, potentially leading to variability of practice. We evaluated the differences in brain death guidelines in major US hospitals with a strong presence of neurology and neurosurgery to determine whether there was evidence of variation from the guidelines as put forth by the American Academy of Neurology (AAN). ⋯ Major differences exist in brain death guidelines among the leading neurologic hospitals in the Unites States. Adherence to the American Academy of Neurology guidelines is variable. If the guidelines reflect actual practice at each institution, there are substantial differences in practice which may have consequences for the determination of death and initiation of transplant procedures.
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The mirror neuron system (MNS) is an observation-execution matching system activated, in humans, during action observation, motor learning, and imitation of action. We used functional MRI (fMRI) to investigate the properties of the MNS in patients with multiple sclerosis (MS). ⋯ This study suggests increased activation of the mirror neuron system in patients with multiple sclerosis (MS) with a normal level of function and widespread CNS damage. The potentialities of this system in facilitating clinical recovery in patients with MS and other neurologic conditions should be investigated.