Myotonic Goats and Migraines

Alan J. McComas

in Galvani’s Spark

Published in print August 2011 | ISBN: 9780199751754
Published online September 2011 | e-ISBN: 9780199897094 | DOI:
Myotonic Goats and Migraines

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Peculiar disorders of muscle, characterized by episodic stiffness or weakness have been known for some time, both in humans and in animals. In 1939 Lindor Brown and McGhee Harvey investigate myotonic goats and show that the stiffness is due to hyperexcitability of the muscle fibres, resulting in repetitive impulse firing. Thirty-five years later, in Cambridge, Shirley Bryant and Richard Adrian deduce, on the basis of microelectrode studies, that the myotonia results from the combination of potassium in the transverse tubules and impermeability of the fibre membrane to chloride ions. In Germany, Frank Lehmann-Horn’s group show that one type of familial periodic paralysis is caused by an inability of the voltage-gated sodium channels to close promptly. Rather later, myasthenia gravis is found by Patrick and Lindstrom to be an autoimmune disorder, with the acetylcholine receptor as the provocative antigen. Finally, a whole series of inherited disorders, ranging from migraine to cardiac arrhythmia, are shown to involve ion channel abnormalities.

Keywords: goats; myotonia; familial paralysis; Lindor Brown; McGhee Harvey; Shirley Bryant; Richard Adrian; Frank Lehmann-Horn; James Patrick; Jon Lindstrom

Chapter.  3678 words.  Illustrated.

Subjects: Neuroscience

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