Surgically treated movement disorders associated with heterotopia

Report of 2 cases

Jeffrey P. Mullin M.D., M.B.A. 2 , Jamie J. Van Gompel M.D. 1 , Kendall H. Lee M.D., Ph.D. 1 , Fredric B. Meyer M.D. 1 and Matt Stead M.D., Ph.D. 3
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  • 1 Departments of Neurosurgery and
  • 3 Neurology, Mayo Clinic, Rochester, Minnesota; and
  • 2 Georgetown University School of Medicine, Washington, DC
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Heterotopic gray matter has been implicated in epilepsy; however, not much is known regarding heterotopia beyond epilepsy. Here, the authors describe 2 pediatric patients with deep heterotopias contiguous with basal ganglia structures. These heterotopias appear to have manifested as movement disorders. One patient presented with a left-sided myoclonus and choreiform movements associated with a right caudate heterotopia; she experienced vast improvement after resection of periventricular heterotopia. The other patient presented with progressive dystonia and a ballistic movement disorder. Initial bilateral globus pallidus internus stimulation resulted in successful treatment of the dystonia; however, her movement disorder worsened. After an extensive workup, including STATISCOM (statistical ictal SPECT coregistered to MR imaging), the patient underwent cortical stimulation with improvement in her movement disorder. To the best of our knowledge, these cases are the first reported instances of heterotopic gray matter associated with movement disorders. Both patients experienced significant improvements following resection of their heterotopias.

Abbreviations used in this paper: DBS = deep brain stimulation; EEG = electroencephalography; GPi = globus pallidus internus; PNH = periventricular nodular heterotopia; SISCOM = subtraction ictal SPECT coregistered to MR imaging; STATISCOM = statistical ISCOM; VEEG = video EEG.

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Contributor Notes

Address correspondence to: Matt Stead, M.D., Ph.D., Mayo 16 West A, 200 First Street SW, Rochester, Minnesota 55905. email: stead.squire@mayo.edu.
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