Deep Brain Stimulation of the Globus Pallidus in a Microcephaly Patient

Jang, Junsoo; Youn, Jinyoung; Lee, Jung-Il; Cho, Kyung Rae

Case report

Journal of the Korean Society of Stereotactic and Functional Neurosurgery 2018;14(2):51-53.
Published online September 30, 2018.

Deep Brain Stimulation of the Globus Pallidus in a Microcephaly Patient

Junsoo Jang, Jinyoung Youn, Jung-Il Lee, Kyung Rae Cho

Department of Neurosurgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea

Abstract

Pallidal stimulation for medically intractable dystonia is known to be effective. However, the optimal target of globus pallidus interna (GPi), so called sweet spot is not clear when the anatomy of deep brain structures are not in ordinary fashion. A 29-yearold woman who presented with severe general dystonia was admitted for deep brain stimulation in our center. The patient was diagnosed with microcephaly at birth. Preoperative magnetic resonance image (MRI) revealed that her GPi was located 17mm lateral from the midplane far medial than the classical coordinate, which is usually 21mm lateral from the midplane. Directional electrodes (St. Jude, Little Canada, MN, USA) were implanted to avoid possible side effects from a narrow effective volume. After optimizing the stimulation direction and amplitudes, her BFMDRS movement and disability scores decreased from 30 to 9 and from 11 to 1, respectively 6 months after surgery. Presentation with a distorted or undeveloped brain, in such case are in need of direct targeting and directional lead stimulation can help increase target precision.

Key Words: Deep brain stimulation, Dystonia, Microcephaly.