J Korean Neurosurg Soc.  2012 Sep;52(3):261-263.

Deep Brain Stimulation of the Globus Pallidus in a 7-Year-Old Girl with DYT1 Generalized Dystonia

• Jin ST
• Lee MK
• Ghang JY
• Jeon SM

Abstract

The experience of pediatric deep brain stimulation (DBS) of the globus pallidus internus (GPi) in the treatment of early-onset DYT1 generalized dystonia is still limited. Here, we report the surgical experience of bilateral GPi-DBS under general anesthesia by using microelectrode recording in a 7-year-old girl with early-onset DYT1 generalized dystonia. Excellent improvement of her dystonia without neurological complications was achieved. This case report demonstrates that GPi-DBS is an effective and safe method for the treatment of medically refractory early-onset DYT1 generalized dystonia in children.

Keyword

Globus pallidus; DYT1; Dystonia; Deep brain stimulation

MeSH Terms

Anesthesia, General
Child
Deep Brain Stimulation
Dystonia
Globus Pallidus
Humans
Microelectrodes

Figure

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  Fig. 1 Postoperative MRI axial T2-weighted (A) and coronal T2-weighted (B) images showing the electrodes within the posteroventral globus pallidus internus.

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  Fig. 2 Preoperative photography of patient showing clinical feature of generalized dystonia (A). Photography of 1 month after surgery shows maintaining of an erect standing position (B).

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  Fig. 3 Preoperative (A) and postoperative (B) photography of patient's hand drawing and writing.

Reference

1. Alterman RL, Tagliati M. Deep brain stimulation for torsion dystonia in children. Childs Nerv Syst. 2007; 23:1033–1040. PMID: 17551738.

2. Augood SJ, Penney JB Jr, Friberg IK, Breakefield XO, Young AB, Ozelius LJ, et al. Expression of the early-onset torsion dystonia gene (DYT1) in human brain. Ann Neurol. 1998; 43:669–673. PMID: 9585364.

3. Constantoyannis C, Berk C, Honey CR, Mendez I, Brownstone RM. Reducing hardware-related complications of deep brain stimulation. Can J Neurol Sci. 2005; 32:194–200. PMID: 16018154.

4. Coubes P, Cif L, El Fertit H, Hemm S, Vayssiere N, Serrat S, et al. Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia : long-term results. J Neurosurg. 2004; 101:189–194. PMID: 15309907.

5. Dekaban AS. Changes in brain weights during the span of human life : relation of brain weights to body heights and body weights. Ann Neurol. 1978; 4:345–356. PMID: 727739.

6. Gross RE, Mewes K, Ghani G. Bakay RA, editor. Anesthesia for movement disorder surgery. Movement Disorder Surgery. The Essentials. 2008. New York: Stuttgart;p. 70–82.

7. Isaias IU, Alterman RL, Tagliati M. Outcome predictors of pallidal stimulation in patients with primary dystonia : the role of disease duration. Brain. 2008; 131:1895–1902. PMID: 18567622.

8. Jeong SG, Lee MK, Kang JY, Jun SM, Lee WH, Ghang CG. Pallidal deep brain stimulation in primary cervical dystonia with phasic type : clinical outcome and postoperative course. J Korean Neurosurg Soc. 2009; 46:346–350. PMID: 19893724.

9. Marks WA, Honeycutt J, Acosta F, Reed M. Deep brain stimulation for pediatric movement disorders. Semin Pediatr Neurol. 2009; 16:90–98. PMID: 19501337.

10. Pinsker MO, Volkmann J, Falk D, Herzog J, Steigerwald F, Deuschl G, et al. Deep brain stimulation of the internal globus pallidus in dystonia : target localisation under general anaesthesia. Acta Neurochir (Wien). 2009; 151:751–758. PMID: 19468677.

11. Vidailhet M, Vercueil L, Houeto JL, Krystkowiak P, Lagrange C, Yelnik J, et al. Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia : a prospective 3 year follow-up study. Lancet Neurol. 2007; 6:223–229. PMID: 17303528.

12. Zorzi G, Marras C, Nardocci N, Franzini A, Chiapparini L, Maccagnano E, et al. Stimulation of the globus pallidus internus for childhood-onset dystonia. Mov Disord. 2005; 20:1194–1200. PMID: 15895426.