Case ReportSuccessful Combination of Pallidal and Thalamic Stimulation for Intractable Involuntary Movements in Patients with Neuroacanthocytosis
Introduction
Neuroacanthocytosis (NA) is specific neurologic symptom of involuntary movements that occur progressively in body and all extremities. We present 2 patients with NA who were treated successfully by combined electrical stimulation of the pallidum and thalamus. Severe involuntary movements lead to disruptions in diet and sleep habits and a deterioration in a person's quality of life. Current treatments are only capable of treating symptoms. There are some reports that deep brain stimulation (DBS) has been used as an experimental treatment in refractory cases of NA, but the outcomes have been variable 1, 5, 6, 9, 10, 13.
Previous studies of DBS in patients with NA report that all involuntary movements were improved by DBS of a single target 1, 5, 6, 9, 10, 13; however, reports indicate that the pallidum and thalamus are the best targets for achieving optimal results 1, 2, 5, 6, 9, 10. The reasons for this discrepancy remain poorly understood because only a small number of patients with NA and a progressive change of involuntary movement have been documented 1, 5, 6, 9, 10, 13. The effect of globus pallidum interna (Gpi) stimulation also remains unclear; however, our experience with stimulation of the ventral oralis complex (Vo complex) in the treatment of severe chorea indicates a promising outcome. We present 2 patients with NA who were treated successfully by combined electrical stimulation of the pallidum and thalamus.
Section snippets
Case 1
Case 1 was 43-year-old man who developed trunk spasms at the age of 35 years, followed by involuntary movements of the tongue and all extremities. Severe involuntary movement of the tongue and lip biting meant that the patient was unable to orally ingest certain foods, and percutaneous endoscopic gastromy was used for daily nutrition. The patient reported mild loss of standing balance and could not walk by himself because of poor balance caused by involuntary movements. Moreover, he experienced
Case 2
Case 2 was a 40-year-old man who developed trunk spasm at 35 years of age, followed by involuntary movement of the upper and lower extremities. Violent movement of the left upper extremity had resulted in injury to the left eyeball, and detachment of the left retina was diagnosed and treated by an ophthalmologist. As a result of this accident, the patient wore special goggles to protect his eye from the choreic movements of the upper extremity. The wearing of these goggles narrowed the visual
Discussion
The 2 cases presented indicate that bilateral DBS to the Gpi and Vo complex can reduce the severity of involuntary movements in patients with NA. This report shows the successful treatment of NA by double targeting of DBS for NA. In our 2 cases, the targets of DBS were the 2 most promising candidates for treatment of involuntary movements. Generally, the Gpi is an effective target for advanced Parkinson disease and primary generalized dystonia, and Gpi stimulation improves abnormal trunk
Acknowlegments
This work was presented in poster form at the 16th Quadrennial Meet0069ng of World Society for Stereotactic and Functional Neurosurgery. May 2013, Tokyo, Japan.
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Conflict of interest statement: The authors declare that the article content was composed in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.