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

Summary 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,...

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Bibliographic Details
Published in:Epilepsy research 2010-05, Vol.89 (2), p.232-237
Main Authors: Canafoglia, Laura, Ciano, Claudia, Visani, Elisa, Anversa, Paola, Panzica, Ferruccio, Viri, Maurizio, Gennaro, Elena, Zara, Federico, Madia, Francesca, Franceschetti, Silvana
Format: Article
Language:English
Subjects:
Adult
Aged
Biological and medical sciences
Cerebral Cortex - physiopathology
Female
Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy
Humans
Lafora body disease
Lafora Disease - diagnosis
Lafora Disease - physiopathology
Male
Malformations of the nervous system
Medical sciences
Middle Aged
Nerve Net - physiopathology
Nervous system (semeiology, syndromes)
Neural Inhibition
Neurology
Paired-pulse protocols
Progressive myoclonus epilepsy
Reaction Time
Time Factors
Transcranial magnetic stimulation
Transcranial Magnetic Stimulation - methods
Unverricht-Lundborg disease
Unverricht-Lundborg Syndrome - diagnosis
Unverricht-Lundborg Syndrome - physiopathology
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Summary:Summary 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.
ISSN:0920-1211
1872-6844
DOI:10.1016/j.eplepsyres.2010.01.006