Restoration of erect posture in idiopathic camptocormia by electrical stimulation of the globus pallidus internus

Report of 2 cases

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The authors report on 2 young patients who developed drug-resistant idiopathic dystonic camptocormia (bent spine) and were treated successfully by deep brain stimulation (DBS) of the globus pallidus internus (GPi). The first patient, a 26-year-old woman, suffered for 3 years from such severe camptocormia that she became unable to walk and was confined to bed or a wheelchair. The second patient, a 21-year-old man, suffered for 6 months from less severe camptocormia; he was able to walk but only for short distances with a very bent spine, the arms in a parallel position to the legs, and the hands almost approaching the floor to potentially support him in case of a forward fall. Within a few days following DBS, both patients experienced marked clinical improvement. At most recent follow-up (44 months in one case and 42 in the other), the patients' ability to walk upright remained normal. Similar findings have only been reported recently in a few cases of camptocormia secondary to Parkinson disease or tardive dyskinesia. On the basis of the experience of these 2 idiopathic cases and the previously reported cases of secondary camptocormia with a favorable response to GPi DBS, the authors postulate that specific patterns of oscillatory activity in the GPi are vital for the maintenance of erect posture and the adoption of bipedal walking by humans.

Abbreviations used in this paper: DBS = deep brain stimulation; DSM-IV-TR = Diagnostic and Statistical Manual of Mental Disorders, fourth edition, text revision; GPi = globus pallidus internus; PD = Parkinson disease.

Article Information

Address correspondence to: Damianos E. Sakas, M.D., Department of Neurosurgery, University of Athens Medical School, Evangelismos Hospital, 4 Marasli Street, 10676 Athens, Greece. email: sakasde@med.uoa.gr.

Please include this information when citing this paper: published online April 9, 2010; DOI: 10.3171/2010.3.JNS09981.

© AANS, except where prohibited by US copyright law.

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Figures

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    Case 1. Electromyograms showing recordings of flexor (rectus abdominus, iliopsoas) and extensor (latissimus dorsi) muscles of the trunk in the supine position (A), upon effort to maintain upright posture (B), and when supported against a wall (geste antagoniste) (C). Note that upon effort to maintain the upright posture, both flexors and extensors contract simultaneously, whereas during the sensory trick maneuver only the extensors contract.

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    Case 1. Photographs obtained before (A) and after (B) the GPi DBS procedure.

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    Case 2. Photographs of the patient upon attempting to walk (A) and (B) when supporting himself against a wall before surgery and walking with erect posture (C) after GPi DBS.

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    Coronal and axial MR images showing the DBS electrode tips lying at the posteroventral GPi, bilaterally.

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