gms | German Medical Science

Objective: Deep brain stimulation (DBS) of certain subcortical areas is a well-established treatment for movement disorders and various other indications. X-linked Dystonia Parkinsonism (XDP) is a rare movement disorder beginning with dystonia and in the follow-up course combined with certain symptoms of Parkinsonism. It is endemic at Panay Island in the Philippines. DBS of the Globus pallidum internum (GPi) is considered in pharmacological refractory cases and worldwide only a few cases are reported. A consecutive series of 16 patients was examined and operated in an interdisciplinary project.

Method: 16 male patients (age: 30-52 years) were considered for DBS surgery and travelled to Germany accompanied by a relative. 13 out of 16 patients were operated in general anaesthesia due to their clinical condition (torticollis etc.). In all patients a stereotactic frame was fixed to the skull and a stereotactic 3D-MRI data set was performed. Target coordinates were related to the mid point of the anterior and posterior commissure: 20 mm lateral, 2 mm anterior and 2 mm inferior. Leads were implanted in the GPi bilaterally. Sequential microrecordings were performed to identify the best definite position. Prior to implantation of the neurostimulator a MRI-scan was performed to check the lead position and rule out complications.

Results: In all patients lead placement was successful and was followed by implantation of the stimulation device. Control MRI revealed in two cases a medial malposition of one lead and lead to replacement to the correct position. In one case a bleeding at the lead trajectory was detected by MRI, but no clinical or neurological signs were evaluated in the patient. A hematoma of the stimulator pocket needed to be evacuated by puncture. All patients showed an improvement of their movement disorder and quality of life, measured with the BFM- or UPDRS-scores, immediate postoperatively and also at follow-up examinations at the Philippines.

Conclusions: DBS of the GPi is a safe and effective treatment option also for patients with XDP-syndromes. A significant improvement of these disabling symptoms can be achieved and leads to an increase of quality of life of those patients. This is the largest patient sample as a prospective series reported with DBS in XDP. Shortly, the long-term results after 12 and 24 months will be evaluated and included in the presentation.