Brain Topography

, Volume 24, Issue 1, pp 65–77 | Cite as

Abnormal ERD/ERS but Unaffected BOLD Response in Patients with Unverricht–Lundborg Disease During Index Extension: A Simultaneous EEG-fMRI Study

  • E. Visani
  • L. Minati
  • L. Canafoglia
  • I. Gilioli
  • A. Granvillano
  • G. Varotto
  • D. Aquino
  • P. Fazio
  • M. G. Bruzzone
  • S. Franceschetti
  • F. Panzica
Original Paper


Electrophysiological studies indicate that Unverricht–Lundborg’s disease (ULD), the most common form of progressive myoclonus epilepsy in Europe, is characterized by the involvement of multiple cortical regions in degenerative changes that lead to enhanced excitation and deficient inhibition. We searched for the haemodynamic correlates of these effects using functional MRI (fMRI) of self-paced index extensions, a well-accepted task highlighting significant differences. EEG and fMRI were simultaneously acquired in 11 ULD patients and 16 controls, performing the index extensions individually (event-related task) as well as repetitively (block task). ERD/ERS analysis was performed for the EEG data in the alpha and beta bands. fMRI time-series were analyzed using the traditional general linear model, as well as with an assumption-free approach, and by means of cross-region correlations representing functional connectivity. In line with the existing literature, ULD patients had enhanced desynchronization in the alpha band and reduced post-movement synchronization in the beta band. By contrast, fMRI did not reveal any difference between the two groups; there were no activation intensity, latency or extent effects, no significant engagement of additional regions, and no changes to functional connectivity. We conclude that, so long as the patients are executing a task which does not induce obvious action myoclonus, the hypothesized abnormalities in pyramidal neuron and interneuron dynamics are relatively subtle, embodied in processes which are not metabolically-demanding and take place at a time-scale invisible to fMRI.


Unverricht–Lundborg disease ERD/ERS analysis Simultaneous EEG-fMRI 



This research has received funding from the European Community’s Seventh Framework Programme (FP7/2007-2013) under grant agreement HEALTH-F5-2008-201076. The authors are grateful to Lucia Salvatoni for outstanding technical assistance during EEG data acquisition. The authors would also like to thank two anonymous reviewers for the insightful feedback they provided on an earlier draft.


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Copyright information

© Springer Science+Business Media, LLC 2010

Authors and Affiliations

  • E. Visani
    • 1
  • L. Minati
    • 2
    • 3
    • 4
  • L. Canafoglia
    • 1
  • I. Gilioli
    • 1
  • A. Granvillano
    • 1
  • G. Varotto
    • 1
    • 5
  • D. Aquino
    • 2
  • P. Fazio
    • 1
  • M. G. Bruzzone
    • 2
  • S. Franceschetti
    • 1
  • F. Panzica
    • 1
  1. 1.Department of NeurophysiologyFondazione IRCCS Istituto Neurologico “Carlo Besta”MilanItaly
  2. 2.Department of NeuroradiologyFondazione IRCCS Istituto Neurologico “Carlo Besta”MilanItaly
  3. 3.Scientific DepartmentFondazione IRCCS Istituto Neurologico “Carlo Besta”MilanItaly
  4. 4.Department of PsychiatryBrighton and Sussex Medical School (BSMS)FalmerUK
  5. 5.Department of BioengineeringPolitecnico di MilanoMilanItaly

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