Short and long interval cortical inhibition in patients with Unverricht-Lundborg and Lafora body disease

Laura Canafoglia, Claudia Ciano, Elisa Visani, Paola Anversa, Ferruccio Panzica, Maurizio Viri, Elena Gennaro, Federico Zara, Francesca Madia, Silvana Franceschetti

Research output: Contribution to journalArticlepeer-review

Abstract

Myoclonus has different clinical and neurophysiological features in patients with Unverricht-Lundborg (ULD) and Lafora body disease (LBD), probably because of a different cortical hyperexcitability profile. To investigate the role of intracortical inhibition in such different presentations, we used paired-pulse transcranial magnetic stimulation (TMS) in ten ULD and five LBD patients, all with a positive molecular diagnosis. All of the patients were treated with antiepileptic drugs (AEDs).In comparison with healthy subjects, both patient groups had significantly defective short intracortical inhibition (SICI), however LBD patients, but not ULD and healthy subjects, had a clear inhibition at ISI 6. ms and ISI 10. ms. Moreover, defective long interval cortical inhibition (LICI) was found in LBD but not ULD patients.The substantial reduction in SICI suggests that both ULD and LBD patients have impaired inhibitory interneuron pools which are involved in the generation of cortical reflex myoclonus, whereas the inhibition found in LBD patients at ISI 6 and 10. ms, as well the reduced inhibition found at long intervals, suggest a more complex circuitry dysfunction possibly involving both excitatory and inhibitory systems. These findings are probably related to the high epileptogenic propensity characterizing LBD with respect to ULD and to the more severely distorted neuronal network resulting from the pathogenesis of LBD.

Original languageEnglish
Pages (from-to)232-237
Number of pages6
JournalEpilepsy Research
Volume89
Issue number2-3
DOIs
Publication statusPublished - May 2010

Keywords

  • Lafora body disease
  • Paired-pulse protocols
  • Progressive myoclonus epilepsy
  • Transcranial magnetic stimulation
  • Unverricht-Lundborg disease

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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