Deep Brain Stimulation of Globus Pallidus Internus in a 16-Year-Old Boy with Severe Tourette Syndrome and Mental Retardation

Abstract

A 16- year-old boy with long-standing severe Tourette syndrome (TS) and mental retardation, non-responsive to complex pharmocological and behavioural treatment was selected for bilateral deep brain stimulation (DBS) of the globus pallidus internus (GPi). Pre-operative and post-operative Yale Tourette syndrome scale (YTSS) scores and several other scores were used to quantify the effect of DBS up to one year follow-up. Although subscores of the YTSS improved, the overall outcome of chronic GPi-DBS showed no substantial therapeutic effect. This finding is in contrast to markedly improved TS of the only two adolescent TS patients in whom DBS has been performed so far. In this article we discuss possible reasons for the poor therapeutic effect of GPi-DBS in our patient contributing to the on-going debate on DBS inclusion criteria for adolescent TS patients.

Key words

deep brain stimulation - globus pallidus internus - Tourette syndrome - treatment - Yale Tourette syndrome scale score

References

• 1 Ackermans L, Temel Y, Cath D. et al . Deep brain stimulation in Tourette's syndrome: Two targets?.  Mov Disord. 2006;  21 709-713
• 2 Bajwa RJ, de Lotbiniere AJ, King RA. et al . Deep brain stimulation in Tourette's syndrome.  Mov Disord. 2007;  22 1346-1350
• 3 Bohlhalter S, Goldfine A, Matteson S. et al . Neural correlates of tic generation in Tourette syndrome: An event-related functional MRI study.  Brain. 2006;  129 2029-2037
• 4 Crews Jr WD, Bonaventura S, Hay CL. et al . Gilles de la Tourette disorder among individuals with severe or profound mental retardation.  Mental retardation. 1993;  31 25-28
• 5 Dehning S, Mehrkens JH, Muller N. et al . Therapy-refractory Tourette syndrome: Beneficial outcome with globus pallidus internus deep brain stimulation.  Mov Disord. 2008;  23 1300-1302
• 6 Flaherty AW, Williams ZM, Amirnovin R. et al . Deep brain stimulation of the anterior internal capsule for the treatment of Tourette syndrome: Technical case report.  Neurosurgery. 2005;  57 E403 ; discussion E403.
• 7 Harcherik DF, Leckman JF, Detlor J. et al . A new instrument for clinical studies of Tourette's syndrome.  J Am Acad Child Psychiatry. 1984;  23 153-160
• 8 Hotes R. The diagnostic and statistical manual of mental disorders DSM IV, 4th edn.. American Psychiatric Association; 2000
• 9 Houeto JL, Karachi C, Mallet L. et al . Tourette's syndrome and deep brain stimulation.  J Neurol Neurosurg Psychiatry. 2005;  76 992-995
• 10 Jankovic J. Tourette's syndrome.  N Engl J Med. 2001;  345 1184-1192
• 11 Kerbeshian J, Burd L. A second visually impaired, mentally retarded male with pervasive developmental disorder, tourette disorder and ganser's syndrome: Diagnostic classification and treatment.  Int J Psychiatry Med. 1986;  16 67-75
• 12 Kerbeshian J, Severud R, Burd L. et al . Peek-a-boo fragile site at 16d associated with Tourette syndrome, bipolar disorder, autistic disorder, and mental retardation.  Am J Med Genetics. 2000;  96 69-73
• 13 Kupsch A, Benecke R, Muller J. et al . Pallidal deep-brain stimulation in primary generalized or segmental dystonia.  N Engl J Med. 2006;  355 1978-1990
• 14 Lawson-Yuen A, Saldivar JS, Sommer S. et al . Familial deletion within nlgn4 associated with autism and tourette syndrome.  Eur J Hum Genet. 2008;  16 614-618
• 15 Lerner A, Bagic A, Boudreau EA. et al . Neuroimaging of neuronal circuits involved in tic generation in patients with Tourette syndrome.  Neurology. 2007;  68 1979-1987
• 16 Long ES, Miltenberger RG. A review of behavioral and pharmacological treatments for habit disorders in individuals with mental retardation.  J Behav Ther Exp Psychiatry. 1998;  29 143-156
• 17 Mink JW, Walkup J, Frey KA. et al . Patient selection and assessment recommendations for deep brain stimulation in tourette syndrome.  Mov Disord. 2006;  21 1831-1838
• 18 Muller N. Tourette's syndrome: Clinical features, pathophysiology, and therapeutic approaches.  Dialogues Clin Neurosci. 2007;  9 161-171
• 19 Nelson EC, Pribor EF. A calendar savant with autism and tourette syndrome. Response to treatment and thoughts on the interrelationships of these conditions.  Ann Clin Psychiatry. 1993;  5 135-140
• 20 Reid AH. Gilles de la Tourette syndrome in mental handicap.  J Ment Defic Res. 1984;  28 ((Pt 1)) 81-83
• 21 Rosenquist PB, Bodfish JW, Thompson R. Tourette syndrome associated with mental retardation: A single-subject treatment study with haloperidol.  Am J Ment Retard. 1997;  101 497-504
• 22 Saito Y, Sugai K, Sasaki M. et al . [A case of congenital hypomyelination neuropathy with type I Chiari malformation and mental retardation].  No to hattatsu. 1997;  29 378-383
• 23 Schneider SA, Robertson MM, Rizzo R. et al . Fragile X syndrome associated with tic disorders.  Mov Disord. 2008;  23 1108-1112
• 24 Searcy E, Burd L, Kerbeshian J. et al . Asperger's syndrome, X-linked mental retardation (MPX23), and chronic vocal tic disorder.  J Child Neurol. 2000;  15 699-702
• 25 Servello D, Porta M, Sassi M. et al . Deep brain stimulation in 18 patients with severe gilles de la tourette syndrome refractory to treatment: The surgery and stimulation.  J Neurol Neurosurg Psychiatry. 2008;  79 136-142
• 26 Shahed J, Poysky J, Kenney C. et al . Gpi deep brain stimulation for tourette syndrome improves tics and psychiatric comorbidities.  Neurology. 2007;  68 159-160
• 27 Tewes U, Schallberger U, Rossmann K. Hamburg-Wechsler-Intelligenztest für Kinder (HAWIK-III). Göttingen Bern, Hogrefe; 1999
• 28 Welter ML, Mallet L, Houeto JL. et al . Internal pallidal and thalamic stimulation in patients with Tourette syndrome.  Arch Neurol. 2008;  65 952-957
• 29 Zarkowska E, Crawley B, Locke J. A behavioural intervention for Gilles de la Tourette syndrome in a severely mentally handicapped girl.  J Ment Defic Res. 1989;  33 ((Pt 3)) 245-253

Correspondence