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The Gilles de la Tourette syndrome: J Child Psychiatry ;35, A population-based epidemiological study of Tourette syndrome among adolescents in Israel. Kerbeshian J, Burd L. The North Dakota prevalence studies of Tourette syndrome and other developmental disorders. Adv Neurol ; Robertson MM, Yakeley J. Gilles de la Tourette syndrome and obsessivecompulsive disorder. Tourette syndrome and obsessive-compulsive disorder. Am J Psychiatry ; Obsessive-compulsive disorder with and without tics in a epidemiological sample of adolescents.

Tics and Tourette syndrome. Mov Disord Society, ; Sensory experiences of Gilles de la Tourette syndrome. Arch Gen Psychiatry ; Gilles de la Tourette syndrome. J Clin Psychiatry ; J Neurol Neurosurg Psychiatry ; Accepted, April 25, Medical treatment of Tourette syndrome is often ineffective or is accompanied by debilitating side effects, therefore prompting the need to evaluate surgical therapies.

We present the case of a year-old woman with severe Tourette syndrome since the age of 10 years. Her symptoms included frequent vocalizations and severe head and arm jerks that resulted in unilateral blindness.

Trials of more than 40 medications and other therapies had failed to relieve the tics. We implanted bilateral electrodes in the anterior limb of the internal capsule, terminating in the vicinity of the nucleus accumbens. At month follow-up, optimal stimulation continued to lower her tic frequency and severity significantly. Our findings suggest that stimulation of the anterior internal capsule may be a safe and effective procedure for the treatment of Tourette syndrome.

The estimated prevalence of TS in the population is 0. Although conventional pharmacological and behavioral therapies can be effective, some patients continue to experience debilitating symptoms. Hence, there is a need for safe alternative treatments that can address both the motor and comorbid psychiatric aspects of the disease. A review of ablative surgeries used to treat TS revealed a diverse set of potential targets, including the frontal lobes, the anterior cingulate gyrus, the thalamus, and the cerebellum, along with the recently reported use of thalamic deep brain stimulation DBS in three patients 8, 9.

Although ablative surgeries have met with varying degrees of success, they are also associated with a significant incidence of morbidity, including cognitive impairment, hemiplegia, dysarthria, akinesia, and worsened tics 8, 9.

Furthermore, lesions are by nature permanent and cannot be adjusted after being created. DBS, in comparison, is adjustable, reversible, and hence offers a significant advantage 9. The anterior limb of the internal capsule AIC subserves limbic system circuitry and contains reciprocal frontothalamic and frontostriatal connections important in motor, cognitive, and emotional function Fig.

Subcaudate tractotomy, an ablative procedure that includes a portion of the ventral striatum, also has been an effective target for the treatment of TS 8. Hence, a reasonable approach was to use a DBS electrode in an effort to target the inferior portion of the AIC and the ventral striatum. Moreover, use of a DBS electrode in this fashion allowed for systematic assessment of the effects of stimulation in both areas Fig.

She was diagnosed at age 10 years. She also experienced frequent vocalizations such as grunts, chirps, and swears. She had sustained limb fractures and repeated retinal detachments resulting from her head movements that ultimately rendered her blind in one eye. Trials of more than 40 medications, from pimozide to tetrabenazine and dronabinol, had been either ineffective or caused significant physical or cognitive impairment.

She also tried biofeedback, relaxation techniques, habit reversal therapy, and botulinum toxin injections without significant improvement. The patient had never had symptoms of OCD, depression, or attention deficit hyperactivity disorder. Formal psychiatric evaluation, neuropsychological testing, and personality testing showed only a mild impulsiveness and a mild distractibility.

The patient had no remarkable past medical or family history. Physical examination revealed no sign of neurological diseases other than TS. Facial sensation and strength were normal, and hearing was intact bilaterally. Movement of the jaw, tongue, and palate was normal. Motor examination demonstrated normal tone, strength, bulk, deep tendon reflexes, coordination, stance, and gain. She also had equal sensation to light touch, vibratory sense, and cold.

Magnetic resonance imaging MRI and positron emission tomography results were unremarkable. Before surgery, her case was reviewed and approved by the Psychiatric Neurosurgery Committee at Massachusetts General Hospital, which is composed of psychiatrists, neurologists, neurosurgeons, an ethicist, and experienced lay people. The patient was advised of the experimental nature of the procedure and signed an informed consent.

Target coordinates were at the midpoint of the anterior limb of the internal capsule, 12 mm lateral from midline and 7 mm below the anterior commissure. As an aid to localization, recordings were made with three tungsten microelectrodes placed in a coronal orientation. Burr holes were placed 3.

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The electrodes were advanced through three cannulas using a motorized microdrive Alpha Omega, Nazareth, Israel. Single-unit activity in the medial and lateral electrodes corresponded to the caudate and putamen, respectively Fig. On several occasions, intraoperative tics seemed to be followed by brief highfrequency neuronal discharges.

However, it was difficult to obtain precise timing of tic onset to confirm this observation. As soon as the recordings were completed and the targets were identified, the recording electrodes were removed and two electrodes Model ; Medtronics, Inc. The electrodes were selected to stimulate both the AIC and the ventral striatum. The electrodes then were connected to a portable stimulator, and the different lead combinations were tested.

One week after the initial procedure, two infraclavicular pulse generators were placed Soletra; Medtronics, Inc. Coronal section of an MRI scan of the anterior internal capsule. Frontal projections to the thalamus, striatum, anterior commissure, and cerebral peduncle are shown as thick solid lines.

Temporal projections to the striatum and the contralateral limbic system are shown as thin solid lines. Projections of the caudate to the lenticular nuclei are shown as thick dashed lines.

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GP, globus pallidus; NA, nucleus accumbens. Her eyesight stabilized after the surgery and she has had no significant limb injuries. Nearly all the clinical effects of stimulation were apparent within a few days after each stimulator adjustment. Several settings produced subjective dysarthria, and one setting, with all contacts negative and the case positive, produced mild rhythmic jaw clenching.

The greatest improvement in symptoms was noted using settings of 4. In addition to the effect on tics, different electrode settings altered her mood profoundly over hours to days. Highvoltage stimulation of the ventral-most contacts, in the vicinity of the nucleus accumbens, produced mild apathy and depression, whereas high-voltage stimulation at the dorsal-most contacts, in the body of the capsule, produced hypomania.

Calaméo - Síndrome de Tourette - Documentação Internet

These effects took hours to days to be apparent. Middle contact stimulation, which helped tics the most, generated a stable euthymic state. Our stimulation adjustments were aimed to attain the optimal tic reduction while maintaining a euthymic state. Because both agitated hypomania and depression were uncomfortable for the patient, and because they posed a theoretical risk of self-injurious behavior, she was not left at such settings for any length of time.

Hence, the patient only spent several days at each of the altered mood states. Stimulation never induced suicidal thoughts or significant disruption of work or home life.

On turning the stimulator off, these effects disappeared over minutes to hours. In several instances, the pulse generator accidentally was turned off. In another instance, the patient experienced a broken connecting wire because of the tics, which was subsequently replaced. In both episodes, the patient noted that her symptoms worsened considerably and requested an assessment of the stimulators.

Several studies have suggested that symptoms of TS stem from dysregulation of limbic-striatal circuits and circuits connecting the orbitofrontal cortex to the medial thalamic nuclei 3, 4, 8.

High-frequency stimulation of these AIC tracts may produce an effect similar to ablative lesions 6. Unlike ablation, however, DBS carries the benefit of being adjustable and is less likely to produce the cognitive impairment previously reported with permanent lesions in this area 8, 9. Given that extensive frontothalamic projections pass through the AIC, DBS in this area indeed may provide a beneficial effect by influencing a similar circuitry.