Low-voltage bilateral pallidal stimulation for severe meige syndrome in a patient with primary segmental dystonia: case report.

Abstract

Meige syndrome (MS) is an adult-onset segmental dystonia characterized by the combination of upper and lower cranial involvement. Its treatment is challenging and the use of oral medication or of botulinum neurotoxin treatment is not decisive. Deep brain stimulation of the globus pallidus internum (GPi DBS) has been used occasionally in severe cases. We report the long-term efficacy of low-voltage chronic bilateral GPi DBS in a patient with segmental dystonia featuring severe MS and cervical brachial involvement. The patient received a bilateral ventroposterolateral GPi implant. Postoperative 3-dimensional reconstruction allowed checking of the definitive position of the electrode and contacts within the targeted nucleus. Following implant, the patient received bilateral low-voltage stimulation, consisting in amplitude of 1.3 V with a pulse width of 90 microseconds and a frequency of 130 Hz, yielding a current of 23 microA. Clinical follow-up for 38 months showed a progressive and sustained improvement of dystonia with unchanged electrical settings throughout the observation period. The patient again undertook normal life activities. Bilateral low-voltage GPi stimulation allowed efficient control of MS symptoms and the associated brachial cervical dystonia.