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How Good at Neurology are You?

Muscular Dystrophy Campaign Muscle and Nerve Centre, Radcliffe Infirmary, Oxford; E-mail: david.hilton-jones{at}clinical-neurology.oxf

EXTRACT

Every time I get the wrong answer in the ‘Test yourself’ section, my belief in subspecialization, for the benefit of our patients, is reinforced. I have hesitated writing, but eventually decided that the recent question on central core disease (Practical Neurology, 2003, 3, 251–253) merited further comment. The take-home message is simple and important – central core disease may be associated with malignant hyperthermia, which is most likely to develop in association with general anaesthesia and which can be fatal. A patient is described whose neuromuscular symptoms were limited to ‘never been great at sport and he was the one who always dropped the ball in his family’, and whose father died unexpectedly during anaesthesia. I appreciate the constraint of space available to the question setters, but three issues require further comment.

Firstly, it is suggested that the ‘best way to confirm the diagnosis’ would be to review the father’s ...

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