TECHNICAL CASE REPORTSDEEP BRAIN STIMULATION OF THE ANTERIOR INTERNALCAPSULE FOR THE TREATMENT OF TOURETTE SYNDROME:TECHNICAL CASE REPORTAlice W. Flaherty, M.D.,Ph.D.Department of Neurology,Massachusetts General Hospital,Harvard Medical School,Boston, MassachusettsZiv M. Williams, M.D.Department of Neurosurgery,Massachusetts General Hospital,Harvard Medical School,Boston, MassachusettsRamin Amirnovin, M.D.Department of Neurosurgery,Massachusetts General Hospital,Harvard Medical School,Boston, MassachusettsEkkehard Kasper, M.D.,Ph.D.Department of Neurosurgery,Massachusetts General Hospital,Harvard Medical School,Boston, MassachusettsScott L. Rauch, M.D.Department of Psychiatry,Massachusetts General Hospital,Harvard Medical School,Boston, MassachusettsG. Rees Cosgrove, M.D.Department of Neurosurgery,Massachusetts General Hospital,Harvard Medical School,Boston, MassachusettsEmad N. Eskandar, M.D.Department of Neurosurgery,Massachusetts General Hospital,Harvard Medical School,Boston, MassachusettsReprint requests:Emad Eskandar, M.D.,Department of Neurosurgery,Massachusetts General Hospital,15 Parkman Street, ACC-331,Boston, MA 02114.Email: [email protected], November 2, 2004.Accepted, April 25, 2005.OBJECTIVE AND IMPORTANCE: Medical treatment of Tourette syndrome is oftenineffective or is accompanied by debilitating side effects, therefore prompting the needto evaluate surgical therapies.CLINICAL PRESENTATION: We present the case of a 37-year-old woman with severeTourette syndrome since the age of 10 years. Her symptoms included frequent vocal-izations and severe head and arm jerks that resulted in unilateral blindness. Trials ofmore than 40 medications and other therapies had failed to relieve the tics.INTERVENTION: We implanted bilateral electrodes in the anterior limb of the internalcapsule, terminating in the vicinity of the nucleus accumbens. At 18-month follow-up,optimal stimulation continued to lower her tic frequency and severity significantly.CONCLUSION: Our findings suggest that stimulation of the anterior internal capsulemay be a safe and effective procedure for the treatment of Tourette syndrome.KEY WORDS: Deep brain stimulation, Internal capsule, Obsessive-compulsive disorder, Tourette syndrome,Ventral striatumNeurosurgery 57[ONS Suppl 3]:ONS-403, 2005 DOI: 10.1227/01.NEU.0000176854.24694.95Tourette syndrome (TS) is characterized byinvoluntary motor and vocal tics and isclosely associated with the spectrum ofobsessive-compulsive disorders (OCDs) (4). Theestimated prevalence of TS in the population is0.5%. Although conventional pharmacologicaland behavioral therapies can be effective, somepatients continue to experience debilitating symp-toms. Hence, there is a need for safe alternativetreatments that can address both the motor andcomorbid psychiatric aspects of the disease. A re-view of ablative surgeries used to treat TS revealeda diverse set of potential targets, including thefrontal lobes, the anterior cingulate gyrus, the thal-amus, and the cerebellum, along with the recentlyreported use of thalamic deep brain stimulation(DBS) in three patients (8, 9). Although ablativesurgeries have met with varying degrees of suc-cess, they are also associated with a significantincidence of morbidity, including cognitive im-pairment, hemiplegia, dysarthria, akinesia, andworsened tics (8, 9). Furthermore, lesions are bynature permanent and cannot be adjusted afterbeing created. DBS, in comparison, is adjustable,reversible, and hence offers a significant advan-tage (9).The anterior limb of the internal capsule (AIC)subserves limbic system circuitry and containsreciprocal frontothalamic and frontostriatal con-nections important in motor, cognitive, andemotional function (Fig. 1) (3). The AIC has beentargeted successfully in the surgical treatment ofOCD (6), and thus provides a potentially usefultarget for TS. Subcaudate tractotomy, an abla-tive procedure that includes a portion of theventral striatum, also has been an effective tar-get for the treatment of TS (8). Hence, a reason-able approach was to use a DBS electrode in aneffort to target the inferior portion of the AICand the ventral striatum. Moreover, use of aDBS electrode in this fashion allowed for sys-tematic assessment of the effects of stimulationin both areas (Fig. 1).CASE REPORTPatient HistoryWe present the results of bilateral electrodeimplantation in the AIC of a 37-year-old right-handed woman with medically intractable TS.She was diagnosed at age 10 years. Her symp-toms at presentation consisted primarily ofNEUROSURGERY VOLUME 57 | OPERATIVE NEUROSURGERY 4 | OCTOBER 2005 | ONS-E403violent retrocollic head jerks and arm movements. She alsoexperienced frequent vocalizations such as grunts, chirps, andswears. She had sustained limb fractures and repeated retinaldetachments resulting from her head movements that ulti-mately rendered her blind in one eye. Trials of more than 40medications, from pimozide to tetrabenazine and dronabinol,had been either ineffective or caused significant physical orcognitive impairment. She also tried biofeedback, relaxationtechniques, habit reversal therapy, and botulinum toxin injec-tions without significant improvement. The patient had neverhad symptoms of OCD, depression, or attention deficit hyper-activity disorder. Formal psychiatric evaluation, neuropsycho-logical testing, and personality testing showed only a mildimpulsiveness and a mild distractibility.The patient had no remarkable past medical or family his-tory. Physical examination revealed no sign of neurologicaldiseases other than TS. Facial sensation and strength werenormal, and hearing was intact bilaterally. Movement of thejaw, tongue, and palate was normal. Motor examination dem-onstrated normal tone, strength, bulk, deep tendon reflexes,coordination, stance, and gain. She also had equal sensation tolight touch, vibratory sense, and cold. Magnetic resonanceimaging (MRI) and positron emission tomography resultswere unremarkable.Before surgery, her case was reviewed and approved by thePsychiatric Neurosurgery Committee at Massachusetts Gen-eral Hospital, which is composed of psychiatrists, neurolo-gists, neurosurgeons, an ethicist, and experienced lay people.The patient was advised of the experimental nature of theprocedure and signed an informed consent.Surgical TechniqueThe ventral aspect of AIC was targeted using stereotacticMRI and computed tomography. Target coordinates were atthe midpoint of the anterior