Advertisement

Longterm deep brain stimulation withdrawal: Clinical stability despite electrophysiological instability

  • Author Footnotes
    1 Shared first authors.
    Diane Ruge
    Correspondence
    Corresponding author. Tel.: +44 2 0 0845 155 5000, fax: +44 20 7278 9836.
    Footnotes
    1 Shared first authors.
    Affiliations
    Sobell Department of Motor Neuroscience and Movement Disorders, UCL-Institute of Neurology, University College London, 33 Queen Square, London WC1N3BG, United Kingdom
    Search for articles by this author
  • Author Footnotes
    1 Shared first authors.
    Laura Cif
    Footnotes
    1 Shared first authors.
    Affiliations
    CHRU Montpellier, Hôpital Gui de Chauliac, Département de Neurochirurgie, Montpellier F-34000 France

    Unité de Recherche sur les Mouvements Anormaux, URMA, France

    INSERM, U661, Montpellier F-34000, France

    Université de Montpellier 1, Montpellier F-34000, France

    CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier F-34000, France
    Search for articles by this author
  • Patricia Limousin
    Affiliations
    Sobell Department of Motor Neuroscience and Movement Disorders, UCL-Institute of Neurology, University College London, 33 Queen Square, London WC1N3BG, United Kingdom
    Search for articles by this author
  • Victoria Gonzalez
    Affiliations
    CHRU Montpellier, Hôpital Gui de Chauliac, Département de Neurochirurgie, Montpellier F-34000 France

    Unité de Recherche sur les Mouvements Anormaux, URMA, France

    INSERM, U661, Montpellier F-34000, France

    Université de Montpellier 1, Montpellier F-34000, France

    CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier F-34000, France
    Search for articles by this author
  • Xavier Vasques
    Affiliations
    IBM France
    Search for articles by this author
  • Philippe Coubes
    Affiliations
    CHRU Montpellier, Hôpital Gui de Chauliac, Département de Neurochirurgie, Montpellier F-34000 France

    Unité de Recherche sur les Mouvements Anormaux, URMA, France

    INSERM, U661, Montpellier F-34000, France

    Université de Montpellier 1, Montpellier F-34000, France

    CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier F-34000, France
    Search for articles by this author
  • John C. Rothwell
    Affiliations
    Sobell Department of Motor Neuroscience and Movement Disorders, UCL-Institute of Neurology, University College London, 33 Queen Square, London WC1N3BG, United Kingdom
    Search for articles by this author
  • Author Footnotes
    1 Shared first authors.

      Highlights

      • DBS can produce prolonged clinical relief outlasting stimulation.
      • This can be the case in a life threatening genetic condition.
      • Despite clinical stability electrophysiological instability occurs when DBS is stopped.
      • Clinic/electrophysiology discrepancy might indicate temporary stability.
      • This should alert clinicians to perform close follow-ups.

      Abstract

      Deep brain stimulation (DBS) is a powerful treatment option for movement disorders, including severe generalised dystonia. After several years of treatment, cases have been reported in which DBS has been stopped without any deterioration in clinical benefit. This might indicate that DBS can restore function in some cases. The mechanism of DBS induced clinical retention effects has been addressed before. Here, the question we asked was if such clinical stability is reflected at the underlying physiology level or whether there is indication to believe that a stand-still of symptoms might be at risk because of neurophysiological instability. We recorded patients with pre-intervention life-threatening or severe genetic dystonia with long lasting clinical benefit when turned off DBS. Despite clinical stability, our physiological studies revealed large changes in the excitability of excitatory and inhibitory motor circuits in the cortex, which exceed normal fluctuation. This discrepancy between instability in the motor network physiology caused by removal of DBS and clinical stability alerts as it potentially indicates a risk to fail and cause symptoms to return.

      Keywords

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'

      Subscribe:

      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Hebb M.O.
        • Chiasson P.
        • Lang A.E.
        • Brownstone R.M.
        • Mendez I.
        Sustained relief of dystonia following cessation of deep brain stimulation.
        Mov Disord. 2007; 22: 1958-1962
        • Ruge D.
        • Cif L.
        • Limousin P.
        • Gonzalez V.
        • Vasques X.
        • Hariz M.I.
        • et al.
        Shaping reversibility? Long-term deep brain stimulation in dystonia: the relationship between effects on electrophysiology and clinical symptoms.
        Brain. 2011; 134: 2106-2115
        • Opal P.
        • Tintner R.
        • Jankovic J.
        • Leung J.
        • Breakefield X.O.
        • Friedman J.
        • et al.
        Intrafamilial phenotypic variability of the DYT1 dystonia: from asymptomatic TOR1A gene carrier status to dystonic storm.
        Mov Disord. 2002; 17: 339-345
        • Nomura Y.
        • Ikeuchi T.
        • Tsuji S.
        • Segawa M.
        Two phenotypes and anticipation observed in Japanese cases with early onset torsion dystonia (DYT1) — pathophysiological consideration.
        Brain Dev. 2000; 22: S92-S101
        • Bentivoglio A.R.
        • Loi M.
        • Valente E.M.
        • Ialongo T.
        • Tonali P.
        • Albanese A.
        Phenotypic variability of DYT1-PTD: does the clinical spectrum include psychogenic dystonia?.
        Mov Disord. 2002; 17: 1058-1063
        • Elkay M.
        • Silver K.
        • Penn R.D.
        • Dalvi A.
        Dystonic storm due to Batten's disease treated with pallidotomy and deep brain stimulation.
        Mov Disord. 2009; 24: 1048-1053
        • Ruge D.
        • Tisch S.
        • Hariz M.I.
        • Zrinzo L.
        • Bhatia K.P.
        • Quinn N.P.
        • et al.
        Deep brain stimulation effects in dystonia: time course of electrophysiological changes in early treatment.
        Mov Disord. 2011 Aug 15; 26: 1913-1921https://doi.org/10.1002/mds.23731
        • Kujirai T.
        • Caramia M.D.
        • Rothwell J.C.
        • Day B.L.
        • Thompson P.D.
        • Ferbert A.
        • et al.
        Corticocortical inhibition in human motor cortex.
        J Physiol. 1993; 471: 501-519
        • Quartarone A.
        • Rizzo V.
        • Terranova C.
        • Morgante F.
        • Schneider S.
        • Ibrahim N.
        • et al.
        Abnormal sensorimotor plasticity in organic but not in psychogenic dystonia.
        Brain. 2009; 132: 2871-2877