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Threshold electrotonus and ion channel dysfunction in motor neurone disease

Cikurel, Katia; (2001) Threshold electrotonus and ion channel dysfunction in motor neurone disease. Doctoral thesis (M.D), UCL (University College London). Green open access

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Threshold electrotonus is a new neurophysiological technique, which records excitability changes in axons induced by subthreshold currents and detects abnormalities in internodal, as well as nodal, nerve membrane. The technique provides a unique means of studying the presumed membrane instability responsible for the generation of fasciculations in motor neurone disease. An initial clinical study using the technique, revealed that eleven patients with motor neurone disease exhibited consistently abnormal changes in excitability produced by 100 ms polarising currents, compared with fifteen normal and nineteen neurological controls. The abnormality was most pronounced 10-20 ms after the onset of the current, suggesting reduced activity of fast and slow potassium channels. The current study was undertaken to assess the reproducibility of these findings and the value of threshold electrotonus as a diagnostic and prognostic tool in motor neurone disease. The study, also, aimed to appraise the effect of riluzole, which has an action on sodium channels, and membrane stabilising medication. Seventy patients with motor neurone disease were studied and were compared to thirty-five normal and sixty-four neurological controls. Thirty-seven of the motor neurone disease patients were followed up every two months for up to two years. The results of the recordings are presented and reveal that although threshold electrotonus is a very specific test for motor neurone disease, it is not as sensitive as was previously expected. Additional data are also presented on the effect of temperature, the use of single unit recordings and the use of the technique in patients with multiple sclerosis, who were found to exhibit a novel abnormality which correlated with disease activity.

Type: Thesis (Doctoral)
Qualification: M.D
Title: Threshold electrotonus and ion channel dysfunction in motor neurone disease
Open access status: An open access version is available from UCL Discovery
Language: English
Additional information: Thesis digitised by ProQuest.
Keywords: Biological sciences; Motor neurone disease; Threshold electrotonus
URI: https://discovery.ucl.ac.uk/id/eprint/10103887
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