Single-Electrode Deep Brain Stimulation of Bilateral Posterolateral Globus Pallidus Internus in Patients With Medically Resistant Lesch-Nyhan Syndrome

Overview

Journal Cureus

Specialty Biomedical Engineering

Date 2023 May 8

PMID 37153246

Authors

Eliza Baird-Daniel

Adam Glaser

Scott Boop

Sharon Durfy

Jason S Hauptman

Affiliations

Soon will be listed here.

Abstract

Deep brain stimulation (DBS) targeting various locations within the globus pallidus internus (GPi) is emerging as a therapeutic option for patients with medically resistant Lesch-Nyhan syndrome. We report our institutional experience with single-electrode DBS in the bilateral posterolateral GPi as an effective method for reduction of both dystonia and self-injurious behavior. Two pediatric patients aged six and 14 years underwent implantation of bilateral singular DBS leads in the posterolateral GPi and were followed postoperatively through the programming process and symptomatic improvements. Caregivers reported that after DBS in the posterolateral GPi, these patients experienced decreased self-mutilation behavior and decreased dystonia.

References

Air E, Ostrem J, Sanger T, Starr P . Deep brain stimulation in children: experience and technical pearls. J Neurosurg Pediatr. 2011; 8(6):566-74. DOI: 10.3171/2011.8.PEDS11153. View

Dabrowski E, Smathers S, Ralstrom C, Nigro M, Leleszi J . Botulinum toxin as a novel treatment for self-mutilation in Lesch-Nyhan syndrome. Dev Med Child Neurol. 2005; 47(9):636-9. View

Taira T, Kobayashi T, Hori T . Disappearance of self-mutilating behavior in a patient with lesch-nyhan syndrome after bilateral chronic stimulation of the globus pallidus internus. Case report. J Neurosurg. 2003; 98(2):414-6. DOI: 10.3171/jns.2003.98.2.0414. View

Serrano M, Perez-Duenas B, Ormazabal A, Artuch R, Campistol J, Torres R . Levodopa therapy in a Lesch-Nyhan disease patient: pathological, biochemical, neuroimaging, and therapeutic remarks. Mov Disord. 2008; 23(9):1297-300. DOI: 10.1002/mds.21786. View

Hale A, Monsour M, Rolston J, Naftel R, Englot D . Deep brain stimulation in pediatric dystonia: a systematic review. Neurosurg Rev. 2018; 43(3):873-880. PMC: 6500764. DOI: 10.1007/s10143-018-1047-9. View

Khasnavis T, Torres R, Sommerfeld B, Garcia Puig J, Chipkin R, Jinnah H . A double-blind, placebo-controlled, crossover trial of the selective dopamine D1 receptor antagonist ecopipam in patients with Lesch-Nyhan disease. Mol Genet Metab. 2016; 118(3):160-166. DOI: 10.1016/j.ymgme.2016.04.012. View

Karachi C, Francois C, Parain K, Bardinet E, Tande D, Hirsch E . Three-dimensional cartography of functional territories in the human striatopallidal complex by using calbindin immunoreactivity. J Comp Neurol. 2002; 450(2):122-34. DOI: 10.1002/cne.10312. View

Cif L, Biolsi B, Gavarini S, Saux A, Robles S, Tancu C . Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease. Mov Disord. 2007; 22(14):2126-9. DOI: 10.1002/mds.21723. View

Lozano A, Lipsman N, Bergman H, Brown P, Chabardes S, Chang J . Deep brain stimulation: current challenges and future directions. Nat Rev Neurol. 2019; 15(3):148-160. PMC: 6397644. DOI: 10.1038/s41582-018-0128-2. View

10.

ANDERSON L, Herrmann L, DANCIS J . The effect of L-5-hydroxytryptophan on self-mutilatin in Lesch-Nyhan disease: a negative report. Neuropadiatrie. 1976; 7(4):439-42. DOI: 10.1055/s-0028-1091644. View

11.

Jinnah H, Ceballos-Picot I, Torres R, Visser J, Schretlen D, Verdu A . Attenuated variants of Lesch-Nyhan disease. Brain. 2010; 133(Pt 3):671-89. PMC: 2842514. DOI: 10.1093/brain/awq013. View

12.

Visser J, Schretlen D, Bloem B, Jinnah H . Levodopa is not a useful treatment for Lesch-Nyhan disease. Mov Disord. 2011; 26(4):746-9. PMC: 3523804. DOI: 10.1002/mds.23478. View

13.

Allen S, Rice S . Risperidone antagonism of self-mutilation in a Lesch-Nyhan patient. Prog Neuropsychopharmacol Biol Psychiatry. 1996; 20(5):793-800. DOI: 10.1016/0278-5846(96)00059-0. View

14.

Nakano K . Neural circuits and topographic organization of the basal ganglia and related regions. Brain Dev. 2000; 22 Suppl 1:S5-16. DOI: 10.1016/s0387-7604(00)00139-x. View

15.

Visser J, Cotton A, Schretlen D, Bloch J, Tedroff K, Schechtmann G . Deep brain stimulation in Lesch-Nyhan disease: outcomes from the patient's perspective. Dev Med Child Neurol. 2021; 63(8):963-968. PMC: 8350791. DOI: 10.1111/dmcn.14852. View

16.

Torres R, Prior C, Puig J . Efficacy and safety of allopurinol in patients with the Lesch-Nyhan syndrome and partial hypoxanthine- phosphoribosyltransferase deficiency: a follow-up study of 18 Spanish patients. Nucleosides Nucleotides Nucleic Acids. 2006; 25(9-11):1077-82. DOI: 10.1080/15257770600893974. View

17.

Visser J, Bar P, Jinnah H . Lesch-Nyhan disease and the basal ganglia. Brain Res Brain Res Rev. 2000; 32(2-3):449-75. DOI: 10.1016/s0165-0173(99)00094-6. View

18.

Lesch M, Nyhan W . A FAMILIAL DISORDER OF URIC ACID METABOLISM AND CENTRAL NERVOUS SYSTEM FUNCTION. Am J Med. 1964; 36:561-70. DOI: 10.1016/0002-9343(64)90104-4. View