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2 Biocybernetics and Biomedical Engineering

2 2012

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Assessment of human motoneuron afterhyperpolarization duration in health and disease

Piotrkiewicz M., Kudina L., Jason Chen J. J., Hausmanowa-Petrusewicz I.

Biocybernetics and Biomedical Engineering

2012

Vol. 32, no. 3

43-61

The results of the investigatigation of afterhyperpolarization (AHP) duration in normal aging and selected neuromuscular disorders are presented. This investigation yielded unexpected results: the AHP shortening in myogenic disease (DMD) and no significant difference from control values in neurogenic disease (ALS). However, introduction of age factor revealed novel aspects of the human ALS, which can be interpreted on the basis of the results obtained in a SOD1 mice, thus confirming usefulness of this animal model of ALS. In spastic patients the AHP was prolonged and the difference from the control AHP duration decreased with age and disease duration. Our results suggest that the match between temporal characteristics of the AHP of MN and of the twitch of its muscle unit is preserved during normal aging and in spasticity, but not in the DMD.

Animal models of duchenne muscular dystrophy, with special reference to the mdx mouse

Blain A., Greally E., Laval S., Blamire A., MacGowan G., Straub V.

Biocybernetics and Biomedical Engineering

2012

Vol. 32, no. 4

3-15

Duchenne muscular dystrophy (DMD) is a progressive muscle wasting disease that affects approximately l in 3500 male births. We describe animal models of DMD with special reference to the mdx mouse. We also describe some of the standard operating procedures (SOPs) developed by the TREAT-NMD neuromuscular network (http://www.treat-nmd. eu/) for assessment of the mdx mouse, with a focus on techniques for assessing cardiac function that are used in our lab, including the cardiac conductance catheter. We have also recently developed cardiac MRI as a novel cardiac assessment technique for mouse models of muscular dystrophy. We describe how this technique can be used both in the assessment of ventricular function and in the investigation of the role of abnormal calcium influx in muscular dystrophy-associated cardiomyopathy.