PMID- 23276489
OWN - NLM
STAT- MEDLINE
DCOM- 20130612
LR  - 20130415
IS  - 1872-8952 (Electronic)
IS  - 1388-2457 (Linking)
VI  - 124
IP  - 5
DP  - 2013 May
TI  - Giant SEPs and SEP-recovery function in Unverricht-Lundborg disease.
PG  - 1013-8
LID - S1388-2457(12)00741-9 [pii]
LID - 10.1016/j.clinph.2012.11.011 [doi]
AB  - OBJECTIVE: To evaluate the relationship between sensory hyperexcitability as 
      revealed by giant SEPs and the SEP recovery function (SEP-R) in a series of 
      patient with progressive myoclonic epilepsy of Unverricht-Lundborg type, 
      identified as epilepsy, progressive myoclonic 1A (EPM1A), MIM #254800. METHODS: 
      We evaluated SEPs by applying median nerve stimuli and SEP-R using paired stimuli 
      at inter-stimulus intervals (ISIs) of between 20 and 600 ms in 25 patients and 20 
      controls. The SEPs were considered "giant" if the N20P25 and P25N33 amplitudes 
      exceeded normal mean values by +3SD. RESULTS: During the paired-stimulus 
      protocol, the SEPs elicited by the second stimulus (S2) were detectable at all 
      ISIs but consistently suppressed in the 13 patients with giant SEPs reflecting a 
      significantly delayed SEP-R. Maximal suppression roughly corresponded to the 
      plateau of a broad middle latency (>100 ms) wave pertaining to the S1 response. 
      CONCLUSIONS: The cortical processing dysfunction generating giant SEPs in EPM1A 
      patients consistently combines with a long-lasting suppression of 
      hyperexcitability that leads to a delayed giant SEP-R without obstructing the 
      response to incoming stimuli. SIGNIFICANCE: The delayed SEP-R is not due to true 
      inhibition but the suppression of aberrant hyper-synchronisation sustaining giant 
      SEPs. A broad middle latency SEP component adds a significantly suppressive 
      effect. This suggests that cortico-subcortical circuitries contribute to both the 
      gigantism and the delayed SEP-R.
CI  - Copyright (c) 2012 International Federation of Clinical Neurophysiology. Published 
      by Elsevier Ireland Ltd. All rights reserved.
FAU - Visani, E
AU  - Visani E
AD  - Department of Neurophysiology, Fondazione IRCCS Istituto Neurologico Carlo Besta, 
      Milan, Italy.
FAU - Canafoglia, L
AU  - Canafoglia L
FAU - Rossi Sebastiano, D
AU  - Rossi Sebastiano D
FAU - Agazzi, P
AU  - Agazzi P
FAU - Panzica, F
AU  - Panzica F
FAU - Scaioli, V
AU  - Scaioli V
FAU - Ciano, C
AU  - Ciano C
FAU - Franceschetti, S
AU  - Franceschetti S
LA  - eng
PT  - Journal Article
DEP - 20121229
PL  - Netherlands
TA  - Clin Neurophysiol
JT  - Clinical neurophysiology : official journal of the International Federation of 
      Clinical Neurophysiology
JID - 100883319
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Electric Stimulation/methods
MH  - Evoked Potentials, Somatosensory/*physiology
MH  - Female
MH  - Humans
MH  - Male
MH  - Median Nerve/*physiopathology
MH  - Middle Aged
MH  - Neural Inhibition/physiology
MH  - Recovery of Function/*physiology
MH  - Unverricht-Lundborg Syndrome/*physiopathology
MH  - Young Adult
EDAT- 2013/01/02 06:00
MHDA- 2013/06/13 06:00
CRDT- 2013/01/02 06:00
PHST- 2012/07/27 00:00 [received]
PHST- 2012/10/22 00:00 [revised]
PHST- 2012/11/17 00:00 [accepted]
PHST- 2013/01/02 06:00 [entrez]
PHST- 2013/01/02 06:00 [pubmed]
PHST- 2013/06/13 06:00 [medline]
AID - S1388-2457(12)00741-9 [pii]
AID - 10.1016/j.clinph.2012.11.011 [doi]
PST - ppublish
SO  - Clin Neurophysiol. 2013 May;124(5):1013-8. doi: 10.1016/j.clinph.2012.11.011. 
      Epub 2012 Dec 29.