We report on four patients in whom muscular pain-fasciculation syndrom
e was diagnosed. Clinical characteristics, electrodiagnostic features
and muscle biopsy findings in our patients were compatible with polyne
uropathy with axonal degeneration. In one case electrophysiological fi
ndings indicated an additional alteration of the first motor neuron. T
he chronic and mild course differentiates this disorder from other neu
romuscular diseases with spontaneous muscle activity. Differential dia
gnosis and pathogenesis of this rare syndrome are discussed on the bas
is of our case records.