Author/Editor		Meznarič-Petruša, M; Vodušek, DB; Zidar, J
Title		Nevromiotonija - opis treh bolnikov
Translated title		Neuromyotonia - report on three cases
Type		članek
Source		Zdrav Vestn
Vol. and No.		Letnik 61, št. 4
Publication year		1992
Volume		str. 207-10
Language		slo
Abstract		Background. The neuromyotonia syndrome includes generalized myokymia and a slowed - down relaxation of the muscles without percussive myotonia. This causes stiffening of the muscles, which does not disappear during sleep. This article analyses the characteristics of three patients and discusses some unclear features connected with the naming of the disease. Methods. For the diagnostics of the disease electromyography, peripheral nerve block and neuromuscular block, and muscular biopsy of the tibialis anterior muscle were used. Results. All three patients exhibited myokymia, two had fasciculations and one had increased muscle tone. Two patients also exhibited absent ancle jerks, and one muscular atrophies and paresis. In the female patient neuromyotonia was the only sign of neuropathy and corresponded to Isaac's syndrome while in the two male patients neuromyotonia was "symptomatic"; associated with hereditary motor and sensory neuropathy of type II in the first and in the second linked with neuropathy and dementia as well as a suspicion of paraneoplastic etiology which, however, was not confirmed. Electromyography revealed characteristic abnormal spontaneous high frequency discharges of motor unit potentials originating in the peripheral nerves. A muscular biopsy confirmed the clinical and electrophysiological signs of neuropathy. Treatment with diphenylhydantoin and carbamazepin had an alleviating effect in all patients. Conclusions. Increased muscle tone can also be a sign of damage to the peripheral motor axone when myokymia and elecof differing genesis. tromyographically, characteristic high freguency discharge of motor unit potentials are established, these being a main feature of neuromyotonia. This is a rare neuromuscular syndrome, which can constitute a disease in itself or can appear within neuropathy.
Descriptors		FASCICULATION ELECTROMYOGRAPHY MUSCLE HYPERTONIA MUSCLES BIOPSY