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
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Article info
Publication history
Published online: May 15, 2014
Accepted:
May 6,
2014
Received in revised form:
May 3,
2014
Received:
March 24,
2014
Identification
Copyright
© 2014 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
