Successful pallidal deep brain stimulation in 15-year-old with Tourette syndrome: 2-year follow-up

Abstract

Dear Sirs,Tourette syndrome (TS) is a potentially disabling chronictic disorder often associated with neuropsychiatric comor-bidities and typical onset during childhood [1]. Medicationfrequently causes bothersome adverse effects and deepbrain stimulation (DBS) has been tried in severe refractorycases [2].A 14-year-old boy with several motor and phonic tics(including coprolalia) since the age of 8 years was referredto surgery due to symptom refractoriness. Pregnancy andbirth were uneventful, and developmental milestones werenormally achieved. Family history was unremarkable,except for one maternal uncle with simple motor tics.Investigations, including uric acid, copper, ceruloplasmin,and brain MRI were normal. Medication was haloperidol8 mg/day and pimozide 8 mg/day. Previously, risperidoneand quetiapine had been tried, as well as habit reversaltherapy and psychoeducational interventions. At this pointseveral motor tics were seen almost continuously, one ofthem causing significant physical injury (see Supplemen-tary online video, captured and published with consentfrom the patient and his mother). Comorbidities includedtroublesome obsessions (e.g. intense fear that he might losecontrol and stab his mother; obsessive concern with con-tagious diseases), compulsions (e.g. compulsive checkingof locks; compulsive re-writing), anxiety and depression. Inthe classroom he found it very difficult to concentrate, andwould get very anxious; constant conflicts with his class-mates occurred, and he was a victim of bullying. Eventu-ally, the patient became unable to attend school or socializenormally, due to embarrassment. Therapeutic optimizationwas tried with clonidine 0.3 mg/day, aripiprazole 20 mg/day, tetrabenazine 50 mg/day, clonazepam 6 mg/day, ser-traline 100 mg/day, and fluvoxamine 200 mg/day, withoutsignificant benefit; higher doses were not tolerated.After careful review of evidence of efficacy and safetywith the multidisciplinary team, bilateral anteromedialinternal pallidal (AM-GPi) DBS was performed at the ageof 15 years. The procedure was approved by the institu-tional ethics committee. Significant benefits were notedimmediately after surgery, with functional and quality oflife (QOL) improvements and no adverse events occurred.There was also significant psychopathological improve-ment, including mood. The patient reported subjectivelylower anxiety levels, and feeling more confident. Table 1details serial assessments, as well as drug therapy andstimulation parameters at each time point (see also Sup-plementary material online). Symptomatic deteriorationwas noted around 1 year after surgery, namely with regardto tics, depression, and QOL. Despite thorough investiga-tion and questioning no life-events or contextual factorscould be related to the worsening. Drug therapy and