Muscle & nerve
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Multicenter Study
Electrophysiological diagnosis and patterns of response to treatment of botulism with neuromuscular respiratory failure.
In this study we describe the electrophysiological findings in botulism patients with neuromuscular respiratory failure from major botulism outbreaks in Thailand. High-rate repetitive nerve stimulation testing (RNST) of the abductor digiti minimi (ADM) muscle of 17 botulism patients with neuromuscular respiratory failure showed mostly incremental responses, especially in response to >20-HZ stimulation. In the most severe stage of neuromuscular respiratory failure, RNST failed to elicit a compound muscle action potential (CMAP) of the ADM muscle. ⋯ Incremental responses to 20-HZ RNST were most useful for diagnosis. The initial electrodiagnostic sign of recovery following treatment of neuromuscular respiratory failure was an increased CMAP amplitude and an incremental response to 10-20-HZ RNST. Muscle Nerve 40: 271-278, 2009.
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Randomized Controlled Trial
Influence of intrinsic noise generated by a thermotesting device on thermal sensory detection and thermal pain detection thresholds.
Various factors can influence thermal perception threshold measurements and contribute significantly to unwanted variability of the tests. To minimize this variability, testing should be performed under strictly controlled conditions. Identifying the factors that increase the variability and eliminating their influence should increase reliability and reproducibility. ⋯ Our results show that thermal sensory thresholds measured with the two devices are comparable. However, our data suggest that, for studies with repeated measurements on the same subjects, a silent thermotesting device may allow detection of smaller differences in the treatment effects and/or may permit the use of a smaller number of tested subjects. Muscle Nerve 40: 257-263, 2009.
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In this article we provide an overview of the intersection between amyotrophic lateral sclerosis (ALS) and the autophagy pathway and discuss the potential protective effects of lithium through mechanisms that recruit autophagy and other effects. The autophagy pathway is recruited during motor neuron (MN) death both in vitro and in vivo. Despite a few controversial issues concerning the significance (detrimental/protective) of autophagy in ALS, recent findings indicate a protective role. ⋯ Lithium also increases the number of Renshaw cells that are affected early during the progression of experimental ALS. Again, lithium has been reported to decrease glial proliferation in the ALS spinal cord and induces sprouting in corticospinal fibers. Muscle Nerve 40: 173-194, 2009.
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The aim of this study was to assess quality of life (QoL) and evaluate the occurrence and characteristics of pain in facioscapulohumeral muscular dystrophy (FSHD) patients. No study has yet assessed QoL in a large group of FSHD patients and, overall, few studies have assessed pain in neuromuscular diseases. We performed a prospective study using a multidimensional protocol including: clinical (according to the Clinical Severity Scale Rev1); genetic (p13E-11 EcoRI fragments Rev1); QoL (Short Form-36); pain (Visual Analog Scale and Portenoy-6 questions); and depression (Beck Depression Inventory) assessment. ⋯ Moreover, we must pay more attention to gender differences: women can suffer far greater deterioration in the emotional aspects of QoL. Further multidimensional observations are needed. Muscle Nerve 40: 200-205, 2009.
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The adductor reflex (AR) is a tendon reflex that has various features that differ from other tendon reflexes. This reflex was tested in different disorders presenting with diminished patellar reflexes such as diabetic lumbosacral radiculoplexus neuropathy (DLRPN), L2-L4 radiculopathy, and distal symmetric diabetic neuropathy (diabetic PNP). The AR and crossed-AR (elicited by tapping the contralateral patellar tendon) were recorded using concentric needle electrodes. ⋯ The latencies of AR, vm-TR, and vm-HR were prolonged in patients with diabetic PNP. We conclude that AR can be useful in the differential diagnosis of some lower motor neuron disorders that present with patellar reflex disturbance. Muscle Nerve 40: 264-270, 2009.