Tolerance of early pallidal stimulation in pediatric generalized dystonia

Report of 2 cases

Restricted access

The authors report on 2 cases of pediatric generalized dystonia with a DYT1 mutation; the patients, an 11-year-old girl and a 9-year-old boy, underwent chronic, pallidal deep brain stimulation (DBS) of the globus pallidus internus (GPi). The dystonic postures in both cases showed dramatic improvements with pallidal DBS, but each patient's symptoms gradually recurred within a year, irrespective of exhaustive readjustments of the stimulation settings. After the recurrence of the dystonic symptoms, the DBS leads were replaced within the GPi in one patient (Case 1) and additional DBS leads were implanted into the bilateral subthalamic nuclei in the other patient (Case 2). Neither measure produced any further clinical benefit, and the patient in Case 2 died of status dystonicus 2 days after reoperation. These findings suggest that early pallidal DBS for pediatric dystonia is indeed effective, although there are some cases in which its therapeutic effect is lost. One possible reason may be the ability of the preadolescent brain to tolerate chronic electrical stimuli during the active maturation process.

Abbreviations used in this paper:BFMDRS = Burke-Fahn-Marsden Dystonia Rating Scale; DBS = deep brain stimulation; GPi = globus pallidus internus; IPG = implantable pulse generator.

Article Information

Address correspondence to: Yasushi Miyagi, M.D., Ph.D., Department of Stereotactic and Functional Neurosurgery, Kaizuka Hospital, 7-7-27 Hakozaki, Higashi-ku, Fukuoka 812-8582, Japan. email: yamiyagi@digital.med.kyushu-u.ac.jp.

Please include this information when citing this paper: published online September 13, 2013; DOI: 10.3171/2013.8.PEDS12578.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Case 1. Axial (A) and coronal (B) MR images showing the location (arrows) of bilateral pallidal electrodes. Line graph (C) demonstrating the serial changes of the motor score of Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS-M; values above the graph). Arrows indicate the surgeries: R = initial pallidal DBS on the right; rp = lateral replacement of the pallidal lead; L = addition of the second pallidal DBS on the left; Lrp = lateral replacement of the pallidal lead; R/L = renewal of the bilateral pallidal leads and extension cables due to lead fracture.

  • View in gallery

    Case 2. Axial (A) and coronal (B) MR images showing the location (arrows) of bilateral pallidal electrodes and subthalamic electrodes. Line graph (C) demonstrating the serial changes of the motor score of the BFMDRS-M (values above the graph). Arrows indicate the surgeries (far left arrows = first pallidal DBS; STN = addition of subthalamic DBS). Open circle and solid line indicate the course of the patient in Case 2; closed circle and dotted line indicate the course of the patient's father.

References

1

Alterman RLMiravite JWeisz DShils JLBressman SBTagliati M: Sixty hertz pallidal deep brain stimulation for primary torsion dystonia. Neurology 69:6816882007

2

Alterman RLTagliati M: Deep brain stimulation for torsion dystonia in children. Childs Nerv Syst 23:103310402007

3

Andrews CAviles-Olmos IHariz MFoltynie T: Which patients with dystonia benefit from deep brain stimulation? A metaregression of individual patient outcomes. J Neurol Neurosurg Psychiatry 81:138313892010

4

Bittar RGYianni JWang SLiu XNandi DJoint C: Deep brain stimulation for generalised dystonia and spasmodic torticollis. J Clin Neurosci 12:12162005

5

Borggraefe IMehrkens JHTelegravciska MBerweck SBötzel KHeinen F: Bilateral pallidal stimulation in children and adolescents with primary generalized dystonia—report of six patients and literature-based analysis of predictive outcomes variables. Brain Dev 32:2232282010

6

Bronte-Stewart HTaira TValldeoriola FMerello MMarks WJ JrAlbanese A: Inclusion and exclusion criteria for DBS in dystonia. Mov Disord 26:Suppl 1S5S162011

7

Cersosimo MGRaina GBPiedimonte FAntico JGraff PMicheli FE: Pallidal surgery for the treatment of primary generalized dystonia: long-term follow-up. Clin Neurol Neurosurg 110:1451502008

8

Cheng JTLiu AWasmuth JLiu BPTruong D: Clinical evidence of genetic anticipation in adult-onset idiopathic dystonia. Neurology 47:2152191996

9

Cif LVasques XGonzalez VRavel PBiolsi BCollod-Beroud G: Long-term follow-up of DYT1 dystonia patients treated by deep brain stimulation: an open-label study. Mov Disord 25:2892992010

10

Coubes PCif LEl Fertit HHemm SVayssiere NSerrat S: Electrical stimulation of the globus pallidus internus in patients with primary generalized dystonia: long-term results. J Neurosurg 101:1891942004

11

Coubes PEchenne BRoubertie AVayssière NTuffery SHumbertclaude V: [Treatment of early-onset generalized dystonia by chronic bilateral stimulation of the internal globus pallidus. Apropos of a case.]. Neurochirurgie 45:1391441999. (Fr)

12

Coubes PRoubertie AVayssiere NHemm SEchenne B: Treatment of DYT1-generalised dystonia by stimulation of the internal globus pallidus. Lancet 355:222022212000

13

Coubes PVayssiere NEl Fertit HHemm SCif LKienlen J: Deep brain stimulation for dystonia. Surgical technique. Stereotact Funct Neurosurg 78:1831912002

14

Goto SYamada KShimazu HMurase NMatsuzaki KTamura T: Impact of bilateral pallidal stimulation on DYT1-generalized dystonia in Japanese patients. Mov Disord 21:178517872006

15

Hamasaki KYamada KHamasaki TKuratsu J: GPi-pallidal stimulation to treat generalized dystonia in Cockayne syndrome. Mov Disord 25:6566582010

16

Haridas ATagliati MOsborn IIsaias IGologorsky YBressman SB: Pallidal deep brain stimulation for primary dystonia in children. Neurosurgery 68:7387432011

17

Iacono RPKuniyoshi SMLonser RRMaeda GInae AMAshwal S: Simultaneous bilateral pallidoansotomy for idiopathic dystonia musculorum deformans. Pediatr Neurol 14:1451481996

18

Jech RBares MUrgosík DCerná OKlement PAdamovicová M: Deep brain stimulation in acute management of status dystonicus. Mov Disord 24:229122922009

19

Katayama YFukaya CKobayashi KOshima HYamamoto T: Chronic stimulation of the globus pallidus internus for control of primary generalized dystonia. Acta Neurochir Suppl 87:1251282003

20

Krause MFogel WKloss MRasche DVolkmann JTronnier V: Pallidal stimulation for dystonia. Neurosurgery 55:136113702004

21

Krauss JK: Deep brain stimulation for dystonia in adults. Overview and developments Stereotact. Funct Neurosurg 78:1681822002

22

Kupsch ABenecke RMüller JTrottenberg TSchneider GHPoewe W: Pallidal deep-brain stimulation in primary generalized or segmental dystonia. N Engl J Med 355:197819902006

23

Kupsch ATagliati MVidailhet MAziz TKrack PMoro E: Early postoperative management of DBS in dystonia: programming, response to stimulation, adverse events, medication changes, evaluations, and troubleshooting. Mov Disord 26:Suppl 1S37S532011

24

Lai TLai JMGrossman RG: Functional recovery after bilateral pallidotomy for the treatment of early-onset primary generalized dystonia. Arch Phys Med Rehabil 80:134013421999

25

Laitinen LVBergenheim ATHariz MI: Ventroposterolateral pallidotomy can abolish all parkinsonian symptoms. Stereotact Funct Neurosurg 58:14211992

26

Lipsman NEllis MLozano AM: Current and future indications for deep brain stimulation in pediatric populations. Neurosurg Focus 29:2E22010

27

Lozano AMKumar RGross REGiladi NHutchison WDDostrovsky JO: Globus pallidus internus pallidotomy for generalized dystonia. Mov Disord 12:8658701997

28

Manji HHoward RSMiller DHHirsch NPCarr LBhatia K: Status dystonicus: the syndrome and its management. Brain 121:2432521998. (Erratum in Brain 123: 419 2000)

29

Mehrkens JHBorggraefe IFeddersen BHeinen FBötzel K: Early globus pallidus internus stimulation in pediatric patients with generalized primary dystonia: long-term efficacy and safety. J Child Neurol 25:135513612010

30

Nomura YIkeuchi TTsuji SSegawa M: Two phenotypes and anticipation observed in Japanese cases with early onset torsion dystonia (DYT1)—pathophysiological consideration. Brain Dev 22:Suppl 1S92S1012000

31

Ondo WGDesaloms JMJankovic JGrossman RG: Pallidotomy for generalized dystonia. Mov Disord 13:6936981998

32

Parr JRGreen ALJoint CAndrew MGregory RPScott RB: Deep brain stimulation in childhood: an effective treatment for early onset idiopathic generalised dystonia. Arch Dis Child 92:7087112007

33

Pinsker MOVolkmann JFalk DHerzog JSteigerwald FDeuschl G: Deep brain stimulation of the internal globus pallidus in dystonia: target localisation under general anaesthesia. Acta Neurochir (Wien) 151:7517582009

34

Pralong EPollo CVillemure JGDebatisse D: Opposite effects of internal globus pallidus stimulation on pallidal neurones activity. Mov Disord 22:187918842007

35

Ruge DTisch SHariz MIZrinzo LBhatia KPQuinn NP: Deep brain stimulation effects in dystonia: time course of electrophysiological changes in early treatment. Mov Disord 26:191319212011

36

Silberstein PKühn AAKupsch ATrottenberg TKrauss JKWöhrle JC: Patterning of globus pallidus local field potentials differs between Parkinson's disease and dystonia. Brain 126:259726082003

37

Starr PATurner RSRau GLindsey NHeath SVolz M: Microelectrode-guided implantation of deep brain stimulators into the globus pallidus internus for dystonia: techniques, electrode locations, and outcomes. J Neurosurg 104:4885012006

38

Tagliati MKrack PVolkmann JAziz TKrauss JKKupsch A: Long-term management of DBS in dystonia: response to stimulation, adverse events, battery changes, and special considerations. Mov Disord 26:Suppl 1S54S622011

39

Taira TKobayashi THori 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 98:4144162003

40

Tisch SRothwell JCLimousin PHariz MICorcos DM: The physiological effects of pallidal deep brain stimulation in dystonia. IEEE Trans Neural Syst Rehabil Eng 15:1661722007

41

Umemura AJaggi JLDolinskas CAStern MBBaltuch GH: Pallidal deep brain stimulation for longstanding severe generalized dystonia in Hallervorden-Spatz syndrome. Case report. J Neurosurg 100:7067092004

42

Vercueil LKrack PPollak P: Results of deep brain stimulation for dystonia: a critical reappraisal. Mov Disord 17:Suppl 3S89S932002

43

Vidailhet MVercueil LHoueto JLKrystkowiak PLagrange CYelnik J: Bilateral, pallidal, deep-brain stimulation in primary generalised dystonia: a prospective 3 year follow-up study. Lancet Neurol 6:2232292007

44

Walcott BPNahed BVKahle KTDuhaime ACSharma NEskandar EN: Deep brain stimulation for medically refractory life-threatening status dystonicus in children. Report of 3 cases. J Neurosurg Pediatr 9:991022012

45

Yianni JBain PGGregory RPNandi DJoint CScott RB: Post-operative progress of dystonia patients following globus pallidus internus deep brain stimulation. Eur J Neurol 10:2392472003

46

Zorzi GMarras CNardocci NFranzini AChiapparini LMaccagnano E: Stimulation of the globus pallidus internus for childhood-onset dystonia. Mov Disord 20:119412002005

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 48 48 12
Full Text Views 57 57 4
PDF Downloads 170 170 8
EPUB Downloads 0 0 0

PubMed

Google Scholar