Firing pattern of fasciculations in ALS: evidence for axonal and neuronal origin.
BACKGROUND
In amyotrophic lateral sclerosis (ALS), the origin of fasciculations is disputed. We hypothesized that the discharge pattern of fasciculation potentials (FPs) would be different for FPs arising in the motor axon or in the spinal motor neuron.


METHODS
FPs were recorded by high-density surface EMG of the biceps brachii or vastus lateralis muscle for 15 minutes in 10 patients with ALS. Records were decomposed into different FP waveforms and their firing moments. Interspike interval (ISI) histograms were constructed for FPs that fired more than 100 times.


RESULTS
Two types of ISI histograms were found. 1) In 23 of 30 different FPs with a total of 8,597 ISIs, the refractory period was 3 to 4 msec. ISIs longer than 15 msec had a Poisson distribution. Five of these 23 FPs discharged doublets with an ISI of approximately 5 msec, indicative of supernormality. This is consistent with the FPs arising in motor axons. 2) In the other 7 FPs, accounting for 11,266 ISIs, the refractory period was 17 to 46 msec. The preferred ISI duration was around 80 msec. Both timing factors are consistent with origin in the spinal motor neuron.


CONCLUSIONS
Firing pattern analysis, based on high-density surface EMG, can detect fasciculation potentials (FPs) of axonal and neuronal origin in amyotrophic lateral sclerosis. The two FP types coexist within the same muscle. The recognition that clinically identical fasciculations conceal the existence of two types of FP that can be studied in a noninvasive manner will introduce a new aspect in the research of motor neuron disease.
Kleine
Bert U
BU
Department of Clinical Neurophysiology, Institute of Neurology, Radboud University Nijmegen Medical Centre, PO Box 9101, 6500HB Nijmegen, The Netherlands. b.kleine@neuro.umcn.nl
Stegeman
Dick F
DF
Schelhaas
Helenius J
HJ
Zwarts
Machiel J
MJ
D000200:Action Potentials / Q000502:physiology*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000175:diagnosis / Q000503:physiopathology*; D001369:Axons / Q000502:physiology*; D004566:Electrodes; D004576:Electromyography / Q000379:methods; D005207:Fasciculation / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology*; D006801:Humans; D008875:Middle Aged; D009046:Motor Neurons / Q000502:physiology*; D011237:Predictive Value of Tests