Objective: We describe and demonstrate by video a patient with dystonia-choreoathetosis cerebral palsy treated with bilateral pallidal deep brain stimulation (DBS).

Background: Dystonia-choreoathetosis cerebral palsy is a motorically disabling form of cerebral palsy. Patients are often refractory to traditional oral medications or have undesirable side effects from the drugs. DBS for the treatment of dystonia has been well established. The treatment of chorea with DBS has been reported with various levels of success. However, reports demonstrating the effects of treating choreoathetosis have been very limited.

Methods: Case Report.

Results: 19 year old man with dystonia-choreoathetosis cerebral palsy secondary to anoxic brain injury at birth, in the setting of labor complicated by uterine rupture, fetal distress and low APGAR scores. At rest, he has intermittent choreoathetoid movements involving head, face, trunk and arms more than legs. Speech is severely dysarthric. Amplitude of choreoathetoid movements increases with speaking, intention and action. He is unable to target, reach, or grasp a cup. Reaching proximally and distally triggers high amplitude coarse overflow chorea and dystonic jerks. Overflow movements also include prominent hip and truncal flexion. Hands and leg are dystonically postured. Finger taps and RAM are slow and uncoordinated. He can stand and walk only with assistance. Bilateral pallidal DBS was implanted. The DBS was programmed with low amplitude, pulse width and frequency which resulted in the reduction in the amplitude of choreic overflow movements. There was less chorea at rest and with action. He was able to feed himself with finger foods. He was also able to hold a cup, and hold a cell phone to his ear.

Conclusions: We report on a young man with dystonia-choreoathetosis cerebral palsy treated with bilateral pallidal DBS. The previously reported cases of dystonia-choreoathetosis cerebral palsy treated with DBS generally show an improvement in dystonia. Interestingly, our patient mainly had improvement in his chorea and large amplitude overflow movements on low DBS setting. Although still significantly limited in his gait and speech, he reported improved control over his arms and hands which allowed him, for the first time, to feed himself. This case is encouraging and adds to the small cohort of patients successfully treated with DBS for dystonia-choreoathetosis cerebral palsy, suggesting that bilateral pallidal DBS is a worthwhile treatment for patients with this condition.

References: Vidailhet, M., Yelnik, J., Lagrange, C., Fraix, V., Grabli, D., Thobois, S., . . . French, S.-S. G. (2009). Bilateral pallidal deep brain stimulation for the treatment of patients with dystonia-choreoathetosis cerebral palsy: a prospective pilot study. Lancet Neurol, 8(8), 709-717. doi:10.1016/S1474-4422(09)70151-6. Krauss, J. K., Loher, T. J., Weigel, R., Capelle, H. H., Weber, S., & Burgunder, J. M. (2003). Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg, 98(4), 785-792. doi:10.3171/jns.2003.98.4.0785.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/improvement-in-chorea-and-function-in-a-patient-with-dystonia-choreoathetosis-cerebral-palsy-after-treatment-with-bilateral-pallidal-deep-brain-stimulation/