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J Neurol (2011) 258:573578 DOI 10.1007/s00415-010-5791-1
ORIGINAL COMMUNICATION
Fasciculations and cramps: how benign? Report of four cases progressing to ALS
Varun Singh John Gibson Brendan McLean
Mike Boggild Nicholas Silver Richard White
Received: 14 July 2010 / Revised: 28 September 2010 / Accepted: 4 October 2010 / Published online: 22 October 2010 Springer-Verlag 2010
Abstract Clinical diagnosis of amyotrophic lateral sclerosis (ALS) in patients presenting with cramps and fasciculations may not be evident at the rst consultation. Sequential reviews, clinical and neurophysiological, form an important part of clinical practice in such cases. Recent attempts to delineate a more benign group with cramps and fasciculations have lacked information on the long term prole, both clinical and neurophysiological. Four patients who were initially diagnosed as suffering from benign cramps and fasciculations, but who subsequently progressed to ALS, are described. We propose that a diagnosis of benign cramps and fasciculations should not be considered secure without a minimum follow up of 45 years.
Keywords ALS Fasciculation Cramps Myokymia
Cramp-fasciculation syndrome
Introduction
The association of fasciculations with cramps is considered an integral part of the clinical presentation of amyotrophic lateral sclerosis (ALS). The disclosure of the diagnosis in those presenting with fasciculations, in view of the invariable fatal outcome of ALS, can only be made after all doubts and uncertainties about the diagnosis have been eliminated. The dilemma facing a clinician whether to make the patient aware of the possibility of the diagnosis is compounded by the observation that cramps and fasciculations are also seen in normal healthy individuals [13]. Questionnaire surveys have established that random muscle twitches [4] and a combination of random twitches and cramps [5] are experienced by healthy subjects. Unfortunately, the long term outcome of fasciculations and cramps either in isolation or combination has never been fully dened in terms of the clinical prole of those who progress to ALS as opposed to those who do not.
The only study addressing this issue [6] involved a telephone survey of subjects identied retrospectively as suffering from fasciculations. One hundred and twenty-one patients were interviewed. The longest duration recorded was 32 years, but whether those with the longest history were one of the 39 cases in whom no fasciculations were recorded on electromyography was not stated. Random...