Aetna considers certain procedures and services medically necessary for assessment and treatment of Tourette's syndrome (TS) when all of the following selection criteria are met.

• Both multiple motor and one or more vocal tics have been present at some time during the illness, although not necessarily simultaneously; and
• The tics occur many times a day (usually in bouts) almost every day or periodically throughout a duration of more than 1 year, and during this period there was never a tic-free period of more than 3 consecutive months; and
• The disturbance causes significant distress or marked impairment in social, occupational, or other important areas of functioning; and
• The onset is before the age of 21 years; and
• The disturbance is not due to direct physiological effects of a substance (e.g., stimulants) or a general medical condition (e.g., Huntington's disease or post-viral encephalitis).

The following procedures and services are considered medically necessary for the assessment and treatment of TS when the selection criteria outlined above are met:

1. Assessment:
   
   
   1. Medical evaluation (complete medical history and physical examination)
   2. Electroencephalography (EEG) or neurological consult (only in the presence of focal signs or clinical suggestions of seizure disorder or degenerative condition)
      
      
2. Treatment:
   
   
   1. Pharmacotherapies*:
      
      
      1. Haloperidol (Haldol and generics)
      2. Pimozide (Orap)
      3. Fluphenazine (Prolixin and generics)
      4. Clonidine (Catapres and generics)
      5. Clonazepam (Klonopin and generics)
      6. Risperidone (Risperdal)
      7. Tricyclic anti-depressants (for TS members who also exhibit attention deficit hyperactivity disorder).
         
         

      * Note: Self-administered prescription medications are covered under the pharmacy benefit. Formulary restrictions may apply. Please check plan benefit description for details.
      
      

   2. Psychotherapy (if member also exhibits anxiety and/or depression).

Note: Psychotherapeutic interventions are covered under the member's behavioral health benefits. Please check benefit plan descriptions.

The following procedures and services are considered experimental and investigational for the assessment and treatment of TS:

1. Assessment:
   
   
   1. Computerized EEG (brain mapping or neurometrics, please see CPB 221 - Quantitative EEG (Brain Mapping))
   2. Neuroimaging (e.g., CT, MRI, PET, and SPECT)
      
      
2. Treatment:
   
   
   1. Botox injections (please see CPB 113 - Botulinum Toxin)
   2. EEG biofeedback (please see CPB 132 - Biofeedback)
   3. Bilateral stereotactic lesions of the anterior cingulate gyrus
   4. Bilateral thalamic stimulation/deep brain stimulation (please see CPB 208 - Deep Brain Stimulation)
   5. Repetitive transcranial magnetic stimulation (please see CPB 469 - Transcranial Magnetic Stimulation and Cranial Electrical Stimulation)
   6. Intravenous immunoglobulins (IVIG) (please see CPB 206 - Intravenous Immunoglobulins (IVIG)).

Note: Most Aetna medical plans exclude coverage of educational interventions. Under these plans, educational and achievement testing as well as educational interventions (including classroom environmental manipulation, academic skills training, and parental training) are not covered. Please check benefit plan descriptions for details.

Tourette's syndrome (TS) is a familial neurobehavioral disorder characterized by fluctuating motor and/or vocal tics. Tics can also be classified as simple or complex. Simple motor tics include eye blinking, neck jerking, shoulder shrugging, and facial grimacing. Complex motor tics include facial gestures, groaning behaviors, hitting or biting oneself, jumping, touching, stamping, and smelling. Simple vocal tics entail coughing, throat clearing, grunting, sniffing, snoring, and barking. Complex vocal tics entail repeating words or phrases out of context, coprolalia (use of obscenities), palilalia (increasingly rapid repetition of a phrase or word), and echolalia (repeating the words of other people). The symptoms of TS generally appear before the patient is 21 years old.

There are no specific blood tests or other laboratory tests that definitively diagnose TS. Diagnosis of this disorder is a clinical one, and is made by patient history, family history, family recounting of events and behaviors of the patient, as well as by direct observation of the patient. A medical evaluation, including a complete history and physical is necessary for diagnosis. According to DSM-IV, both motor and vocal tics must be present for at least 1 year to establish a diagnosis of TS. Brain mapping (computerized EEG) as well as neuroimaging studies, such as computed tomography, magnetic resonance imaging, positron emission tomography, and single photon emission computed tomography, usually do not aid in the diagnosis of TS. Adams et al (2004) stated that the cause or causes of TS remain unknown. Functional imaging studies have evaluated several implicated neurotransmitter systems and focused predominantly on the frequency or severity of tics. The results have been inconclusive and frequently contradictory with little light shed on pathogenetic mechanisms. Electroencephalography or neurological consultation is indicated only in the presence of focal signs or clinical suggestions of seizure disorder or degenerative condition.

For most patients with TS, the clinical course is benign. For patients whose symptoms interfere with daily functioning, pharmacotherapy may help in alleviating symptoms. The most commonly used medications for the treatment of TS are haloperidol (Haldol), pimozide (Orap), fluphenazine (Prolixin), and clonidine (Catapres). Clonazepam (Klonopin) and risperidone (Risperdal) have been shown to be beneficial in some patients. Furthermore, tricyclic anti-depressants can be used for the treatment of TS patients who also exhibit attention deficit hyperactivity disorder. Psychotherapy is appropriate for TS patients who experience anxiety or depression.

There is insufficient scientific evidence to support the use of Botox (botulinum toxin) injections, biofeedback, bilateral stereotactic lesions of the anterior cingulate gyrus, bilateral thalamic stimulation, intravenous immunoglobulins and repetitive transcranial magnetic stimulation for the treatment of TS.

Maciunas et al (2007) performed a prospective double-blind crossover trial of bilateral thalamic deep brain stimulation (DBS) in 5 adults with TS. An independent programmer established optimal stimulator settings in a single session. Subjective and objective results were assessed in a double-blind randomized manner for 4 weeks, with each week spent in one of four states of unilateral or bilateral stimulation. Results were similarly assessed 3 months after unblinded bilateral stimulator activation while repeated open programming sessions were permitted. In the randomized phase of the trial, a statistically significant (p < 0.03) reduction in the modified Rush Video-Based Rating Scale score (primary outcome measure) was identified in the bilateral on state. Improvement was noted in motor and sonic tic counts as well as on the Yale Global Tic Severity Scale and TS Symptom List scores (secondary outcome measures). Benefit was persistent after 3 months of open stimulator programming. Quality of life indices were also improved; 3 of 5 patients had marked improvement according to all primary and secondary outcome measures. The authors concluded that bilateral thalamic DBS appears to reduce tic frequency and severity in some patients with TS who have exhausted other available means of treatment. The findings of this study need to be validated by longer studies with larger sample sizes.

Visser-Vandewalle (2007) noted that following the introduction of DBS of the thalamus as a new treatment for TS in 1999, several other brain loci (e.g., globus pallidus internus, anteromedial and ventroposterolateral part, and the nucleus accumbens) have been targeted in a small number of patients. In published reports, a tic reduction rate of at least 66 % has been described. The effects of DBS on associated behavioral disorders are more variable. The number of treated patients is small and it is unclear if the effects of DBS are dependent on the target nucleus. The author stated that a meticulous evaluation of the electrode position, and a blinded assessment of the clinical effects on tics and behavioral disorders, is absolutely mandatory in order to identify the best target of DBS for TS.