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.
Damianos E. Sakas, Ioannis G. Panourias, Lampis C. Stavrinou, Efstathios J. Boviatsis, Marios Themistocleous, Pantelis Stathis, George Tagaris, Elias Angelopoulos, and Stylianos Gatzonis
Damianos E. Sakas, Lampis C. Stavrinou, Efstathios J. Boviatsis, Pantelis Stathis, Marios Themistocleous, and Stylianos Gatzonis
Dystonia is a movement disorder notoriously difficult to treat. While primary dystonia is classically considered to respond well to deep brain stimulation (DBS), treatment of secondary dystonia yields variable results. Patient selection should be done on a case-by-case basis. Clearly, there is a need to accumulate additional information with regard to prognostic factors that may aid neurosurgeons in selecting those patients in whom the disorder is most likely to respond favorably to pallidal DBS.
The authors report the case of a 29-year-old man with secondary dystonia due to perinatal hypoxia. The most prominent symptom was what we have termed ectatocormia—that is, severe, fixed truncal hyperextension and retrocollis, exacerbated by phasic, twisting movements of the trunk and head. This made it impossible for the patient to maintain a normal upright posture or to walk. The patient underwent bilateral DBS of the globus pallidus internus (GPi), and the authors observed impressive improvement in motor abilities and function. The patient's body adopted the normal upright posture and he became able to walk again, 4 months after the commencement of GPi stimulation.
This report, along with others, emphasizes that the GPi as an ideal target for alleviating axial tonic symptoms. The presence of normal MR imaging findings, a phenotypical purity of predominantly dystonic symptoms, and a younger age seem to favor a positive outcome.