Ahead of Print: Intraventricular versus Intrathecal Baclofen

Background: Intrathecal baclofen (ITB) is an effective treatment for secondary dystonia. However, in many patients with dystonia, placement of an intrathecal catheter is difficult due to anatomic anomalies or spinal fusion. Intraventricular baclofen (IVB) has been shown to be an effective alternate route for drug delivery in a small series of patients.

Objective: To present the largest series of IVB cases to date, and compare the complication rateto that of a concurrent cohort of ITB cases.

Methods: The senior author’s series of IVB cases were reviewed. All contemporaneous cases of ITB for dystonia were reviewed as a control group. Data were collected by retrospective medical records review.

Ahead of Print: Meige Syndrome Relieved by Bilateral Pallidal Stimulation with Cycling Mode

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BACKGROUND AND IMPORTANCE: Deep brain stimulation (DBS) of bilateral globus pallidus internus (GPi) has been used effectively in the treatment of dystonia. We report a patient with severe Meige syndrome who received bilateral GPi DBS with good improvement in symptoms during the first 24-month stimulation therapy. In order to decrease energy consumption and to prolong battery life, the stimulation parameters of the replaced programmable pulse generator (IPG) were adjusted to the cyclic mode in combination with the stimulator turned-off during night time sleep. The patient achieved similar good treatment effect with extended battery use duration in the following years.

CLINICAL PRESENTATION: A 66-year-old woman with a 3-year history of severe cranial-cervical dystonia received stereotaxic surgery for bilateral GPi DBS therapy. The Fahn-Marsden (BFM) dystonia score improved from 32 to 7.5. The effect lasted up to 24 months after therapy when the battery ran out of life. After careful evaluation, we adjusted the stimulation parameters of the second implantable pulse generator to cyclic stimulation mode and programmed the stimulator to turn-off automatically during night time sleep. The patient shows persistent good effect 36-months after starting use of the second IPG.

Video: Low-Voltage GPi-DBS for Meige’s Syndrome

This is a pre-operative video of a patient who developed a progressive bilateral blepharospasm in 1993, at age of 56. By 1994, he developed involuntary lower face contractions and severe tongue spasms associated with difficulties in speech articulation and swallowing. A diagnosis of MS 1 was then formulated. There was no evidence of a somatoform or psychogenic disorder. Brain MRI was unremarkable. By 2001, dystonia gradually spread to further involve the cervical district. Upon examination in 2005, the patient presented with cranial and cervical dystonia, encompassing severe blepharospasm, oromandibular dystonia with prominent tongue protrusion, cervical dystonia with left head tilt and rotation to the left, proximal and distal upper limb dystonia. He also complained of temporo-mandibular pain, dysarthria and dysphagia. Gestes antagonistes consisted in touching his chin with the right hand index and thumb and in chewing a gum to facilitate speaking and swallowing.

First International Congress on Treatment of Dystonia

Registration is still open to attend the First International Congress on Treatment of Dystonia. The conference, taking place May 6-8 in Hannover, Germany, consists of workshops, forums and lectures for dystonia experts and caretakers.

Dystonia is a movement disorder that can be helped with deep brain stimulation. The goal of this meeting is to advance existent therapies, improve their differential use and stimulate research in new therapeutic approaches.