Low-rate nerve stimulation during regional ischemia in the diagnosis of muscle glycogenosis

Mauro Lomonaco, Margherita Milone, Enza Maria Valente, Luca Padua, Pietro Tonali

Research output: Contribution to journalArticlepeer-review

3 Scopus citations


Five patients with muscle glycogenoses (Gly), 30 normal subjects (NS), and 52 disease controls received 3 Hz repetitive stimulation of the ulnar nerve for 4 min (P-LRNS) during regional ischemia and during normal circulation. During regional ischemia, the compound muscle action potential (CMAP) of NS showed 8.5% (SE: 1.0) facilitation after 1 min of P-LRNS and 4.8 ± 1.5% facilitation after 4 min. Gly showed 8.1 ± 1.0% facilitation after 1 min of P-LRNS but -58.6 ± 6.9% depression after 4 min of P-LRNS. During normal circulation, in 18 NS and 4 Gly tested, the facilitation detected after 1 min of P-LRNS was unchanged until the end of stimulation. Compared with the mean ± 2 SD of NS, individual values of CMAP depression were abnormal in all Gly patients already by the 3rd min of ischemic P-LRNS. Of disease controls, myasthenia gravis patients only showed a CMAP depression during ischemic P-LRNS which was distinguishable from that detected in Gly, being mainly induced by neuromuscular transmission block.

Original languageEnglish (US)
Pages (from-to)1523-1529
Number of pages7
JournalMuscle and Nerve
Issue number12
StatePublished - Dec 1996


  • compound muscle action potential depression
  • compound muscle action potential facilitation
  • muscle glycogenosis
  • regional ischemia
  • repetitive nerve stimulation

ASJC Scopus subject areas

  • Physiology
  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)


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