Peripheral (limb) myokymic discharges in motor neuron disease

Abstract

SummaryWe present the case of a 28-year-old gentleman who presented with weakness and wasting in the right arm. He complained about painful cramps in his left leg but there were no sensory, visual or swallowing problems. Neurological examination was significant for asymmetric weakness of both upper and lower limbs and deep tendon reflexes were asymmetrically brisk. Initial laboratory investigations, MRI brain and MRI spine were normal. Electromyography revealed active denervation and chronic neurogenic motor unit action potential. Myokymic discharges were noted in the left deltoid muscle. BACKGROUND

EMG-Phänomene peripherer motorisch axonaler Übererregbarkeit

“…Myokyme Entladungen finden sich unspezifisch bei einer Reihe von neurologischen Erkrankungen, wie etwa bei Motoneuronerkrankungen, antikörpervermittelten Erkrankungen spannungsabhängiger Kaliumkanäle (Isaacs Syndrom), episodischer Ataxie mit Myokymien, Multipler Sklerose, pontinen Gliomen, Hirnnervenschädigungen, demyelinisierenden Polyneuropathien, strahleninduzierten peripheren Nervenverletzungen, Karpaltunnelsyndrom, Syringobulbie, multipler Systematrophie oder etwa unter dem Einfluss des Toxins der Klapperschlange 14 45 46 47 50 51 54 55 56 57 58 59 60 61 62 63 64 65 66 67 68 69 70 71 72 73 74 75 76 77 78 79 .…”