Medical Policy

 

Subject: Deep Brain, Cortical, and Cerebellar Stimulation
Document #: SURG.00026 Publish Date:    05/10/2018
Status: Revised Last Review Date:    05/03/2018

Description/Scope

This document addresses the use of deep brain, cortical, and cerebellar stimulation. These technologies involve the use of high-frequency electrical stimulation of a specific site on or within the brain via implanted unilateral or bilateral electrodes that are connected to a pulse generator.  For deep brain stimulation (DBS) and cerebral stimulation, the generator may be implanted in the chest.  For cortical stimulation, the generator may be implanted in the head.  These forms of electrical stimulation are used in the treatment of intractable movement disorders characterized by involuntary tremors or muscle contractions as well as for seizure disorders.

Position Statement

Medically Necessary:

Unilateral or bilateral deep brain stimulation is considered medically necessary for individuals with disabling, medically unresponsive Parkinson's disease who meet the following criteria:

  1. A minimal score of 30 points on the motor portion of the Unified Parkinson's Disease Rating Scale when the individual has been without medication for 12 hours; and  
  2. Motor complications of therapy that cannot be controlled pharmacologically; or
  3. Individuals with medically refractory tremor from Parkinson’s disease.

Unilateral or bilateral deep brain stimulation is considered medically necessary for individuals with medically refractory essential tremor.

Unilateral or bilateral deep brain stimulation of the subthalamic nucleus or globus pallidus is considered medically necessary for individuals who are seven (7) years of age and older with primary dystonia and who have ALL of the following:

  1. Dystonia is chronic, refractory to drugs, and has a significant effect upon daily activity; and
  2. Dystonia is not due to a secondary cause such as stroke, cerebral palsy, tumor, trauma, infection, multiple sclerosis, other neurodegenerative diseases, or medications; and
  3. Dystonia manifests as one or more of the following:
    1. Cervical dystonia (torticollis); or
    2. Segmental dystonia; or
    3. Generalized dystonia; or
    4. Hemidystonia.

The use of cortical stimulation is considered medically necessary for individuals with epilepsy who have met the criteria below:

  1. 18 years of age or older; and
  2. Partial onset seizures; and
  3. Undergone diagnostic testing that localized no more than two (2) epileptogenic foci; and
  4. Refractory to two or more antiepileptic medications; and
  5. Currently having an average of three (3) or more disabling seizures (for example, motor partial seizures, complex partial seizures, or secondary generalized seizures) per month over the most recent three months.

Investigational and Not Medically Necessary:

Deep brain stimulation for tremor and dystonia from other causes such as trauma, multiple sclerosis (MS), degenerative disorders, metabolic disorders, infectious diseases, and drug-induced movement disorders is considered investigational and not medically necessary.

Deep brain stimulation is considered investigational and not medically necessary for all other conditions not identified as medically necessary, including, but not limited to, the treatment of epilepsy, chronic cluster headache, obsessive-compulsive disorder (OCD) and Tourette syndrome.

The use of cerebellar stimulation/pacing is considered investigational and not medically necessary.

The use of cortical stimulation is considered investigational and not medically necessary for all other indications, including but not limited to individuals who have not met the medically necessary criteria above.

Rationale

Deep Brain Stimulation for Dystonia, Parkinson’s Disease, and Tremor

A variety of randomized studies have shown that deep brain stimulation (DBS) in various locations such as the globus pallidus, subthalamic nucleus or thalamus improved the symptoms of medically refractory Parkinson’s disease compared either to sham stimulation or pallidotomy.  Additionally, randomized controlled studies have shown that deep brain stimulation of the thalamus improves the symptoms of essential tremor compared to sham stimulation (Deuschl, 2000; Figuerias-Mendez, 2002; Merello, 1999; Obeso, 2001; Rehncrona, 2003; Schuepbach, 2013).  DBS has become a standard treatment for cases of Parkinson’s disease when they become refractory to medical therapy.

Primary (or idiopathic) dystonia is dystonia that is not due to a secondary cause such as stroke, cerebral palsy, tumor, trauma, infection, multiple sclerosis, medications, or a neurodegenerative disease.  In 1997, the Activa® Dystonia Therapy System (Medtronic, Minneapolis, MN) received Pre-Market Approval (PMA) from the U.S. Food and Drug Administration (FDA) for unilateral thalamic stimulation for the suppression of tremor in the upper extremity in individuals who are diagnosed with essential tremor or parkinsonian tremor not adequately controlled by medications and where the tremor constitutes a significant functional disability (Roper, 2016; Tan, 2016).  In 2003, this system was granted a Humanitarian Devices Exemption (HDE) by the FDA for the treatment of primary dystonia.  The FDA’s decision was based on the results of deep brain stimulation in 201 individuals represented in 34 manuscripts.  There were three studies that reported at least 10 cases of primary dystonia.  In these studies, clinical improvement ranged from 50% to 88%.  A total of 21 children were studied; 81% were older than 7 years.  Among these individuals there was about a 60% improvement in clinical scores.  The FDA analysis of risk and probable benefit indicated that the only other treatment options for chronic refractory primary dystonia are neurodestructive procedures and DBS provides a reversible alternative.

The FDA Summary of Safety and Probable Benefit states:

Limited treatment strategies exist for chronic, intractable (drug refractory) primary dystonia, including generalized and/or segmental dystonia, hemidystonia, and cervical dystonia (torticollis). The three main approaches to the treatment of primary dystonia include systemic pharmacological agents (oral medications), local pharmacological agents (injected directly into affected muscles or their nerve supply), and destructive surgical or neurosurgical intervention. When local injection therapy is impractical or unsafe, and when systemic medications are not effective or produce unacceptable side effects, surgery may be considered. Surgical treatments of dystonia, including ablative therapies such as thalamotomies and pallidotomies, are irreversible, destructive procedures that can be associated with disabling complications. The patient group characterized in the Humanitarian Use Device application may also be candidates for deep brain stimulation therapy. Although there are a number of serious adverse events experienced by patients treated with deep brain stimulation, in the absence of therapy, chronic intractable dystonia can be very disabling and in some cases, progress to a life threatening stage or constitute a major fixed handicap. When the age of dystonia occurs prior to the reaching their full adult size, the disease not only can affect normal psychosocial development (due to ostracization and/or prevention of normal peer relationships), but also cause irreparable damage to the skeletal system. As the body of the individual is contorted by the disease, the skeleton may be placed under constant severe stresses which may cause permanent disfigurement.

Risks associated with DBS therapy for dystonia appear to be similar to the risks associated with the performance of stereotactic surgery and the implantation of DBS systems for currently approved indications (Parkinson’s Disease and Essential Tremor), except for when used in either child or adolescent patient groups. These additional risks include the use of general anesthetic instead of local anesthesia during implantation, potential lead strains or fractures related to elongation of the trunk of the patient (due to normal growth) while the length of implanted conductor (from the neurostimulator to the burr hole) remains fixed, the risk of lead migration due to patient head growth resulting in ineffective stimulation and the added risk of children being engaged in active play and sports activities that could damage components of the implanted system. The risks of lead strain, fracture and migration can be minimized by evaluating the patient’s implanted lead/extension assembly for sufficient strain relief at regular post-implant follow-up sessions and by considering the replacement of the extension with one of greater length during other elective surgery procedures, such as during the regular change out of neurostimulators that must occur because of battery depletion. In cases where lead tip displacement may occur due to cranial growth the lead tip migration may be accommodated through reprogramming due to the number and spacing of the electrode contacts.

Therefore, it is reasonable to conclude that the probable benefit to health from using the device for the target population outweighs the risk of illness or injury, taking into accounts the probable risks and benefits of currently available devices or alternative forms of treatment when used as indicated in accordance with the directions for use.

Volkmann and colleagues published the results of a randomized controlled trial (RCT) involving 40 subjects with severe generalized or segmental idiopathic dystonia.  Subjects were assigned to either sham or active neurostimulation of the internal globus pallidus.  After implantation of the stimulation device in both groups, the experimental group received active treatment for 9 months.  The control group had an initial 3-month period with their implanted device deactivated (sham treatment), followed by 6 months of active treatment.  The initial intention of the study was to cease follow-up at the end of 9 months, but was extended to 5 years.  Of the initial 40 subjects, 32 (80%) completed the 5-year study period.  In an intention-to-treat analysis including all subjects, significant improvements were reported in dystonia severity at 3 years and 5 years compared with baseline (p=0.001 for both time periods).  In the per-protocol population, results on the Burke–Fahn–Marsden Dystonia Rating Scale (BFMDRS) II motor score from 6 months to 3 years were statistically significant (p=0.001) and were sustained at the 5 year follow-up.  A progressive improvement of dystonia severity beyond 6 months of neurostimulation was predominantly seen in subjects with generalized dystonia.  Subjects with segmental dystonia demonstrated a relatively stable status from 6 months through 5 years.  With the exception of speech and swallowing, all motor symptoms as well as the global clinical assessments of dystonia and pain showed significant improvements for up to 5 years.  The responder analysis showed a beneficial response in 83% (30/36) of subjects at 6 months, 94% (29/31) at 3 years, and 81% (26/32) at 5 years.  Use of antidystonic drugs gradually tapered off for most subjects, decreasing from 60% (20/40) at baseline to 35% (15/40) at 6 months.  This improvement remained relatively constant, with 45% (14/31) of subjects on pharmacotherapy at 3 years and 8% (9/32) at 5 years.  Dysarthria (n=16) and transient worsening of dystonia (n=7) were the most common non-serious adverse events.  Twenty-one subjects experienced serious adverse events that required hospital admission.  Sixteen of the 21 serious adverse events were device-related and were caused by technical defects, delayed infection, or migration.  All serious adverse events in the original 9-month study phase and 66.6% of events during the long-term extension occurred in subjects with generalized dystonia.  Fourteen of the 16 events of dysarthria occurred in subjects with segmental dystonia.  This study demonstrates significant benefits of DBS in individuals with dystonia.  However, substantial differences were found in outcomes between subjects with generalized vs. segmental dystonia.  Further investigation into this issue is warranted.

Volkmann (2014) reported the results of a double-blind RCT involving 62 subjects with cervical dystonia assigned to receive either palladial neurostimulation (n=32) or sham stimulation (n=30).  Data were available for 60 subjects (97%) at 3 months and 56 subjects (90%) at 6 months.  At 3 months, the reduction in dystonia severity as measured with the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) was significantly improved in the experimental group vs. controls (-5.1 points vs. 1.3; p=0.0024).  Adverse events were reported in 11 experimental group subjects (21 events), with 5 events considered serious. The authors reported 11 serious adverse events (5 experimental group subjects vs. 6 controls), including infection, device explantation, and electrode dislocation.  The results of this trial are promising, and demonstrate a significant benefit to DBS therapy in individuals with cervical dystonia.

On June 12, 2015, the FDA granted PMA approval for the Brio Neurostimulation system to reduce symptoms of Parkinson’s disease and essential tremor in individuals with symptoms not adequately controlled with drug therapy.  According to the FDA, data supporting the safety and effectiveness of the device included two clinical studies.  The first included 136 subjects with Parkinson’s disease followed for 3 months following device implantation.  The second included 127 subjects with essential tremor followed for 6 months.  Both studies demonstrated statistically significant improvement in the primary effectiveness endpoints when the device was turned on compared to when it was turned off.

Deep Brain Stimulation for Obsessive Compulsive Disorder

On February 19, 2009, the Reclaim™ device (Metronic Neuro, Minneapolis, MN) received FDA approval under the Humanitarian Devices Exemption (HDE) process.  The FDA labeling states that the device is indicated for bilateral stimulation of the anterior limb of the internal capsule (AIC), as an adjunct to medications and as an alternative to anterior capsulotomy for treatment of chronic, severe, treatment-resistant OCD in adults who have failed at least three selective serotonin reuptake inhibitors (SSRIs).

The use of DBS for OCD has been studied in multiple RCTs, but the vast majority of them involved 10 or fewer subjects.  At this time, only three studies involve larger populations.

As part of a clinical trial, Mallet and colleagues (2008) reported preliminary findings of a crossover, double-blind, multicenter study of DBS for treatment of refractory obsessive-compulsive disorder (OCD).  Eighteen individuals were enrolled, 1 withdrew and 1 required removal of the stimulator before randomization because of infection.  Three months after surgery, 8 individuals were randomly assigned to receive active stimulation for 3 months, followed by 1 month of washout, then 3 months of sham stimulation (on-off group).  The other group followed the same treatment schedule in reverse (off-on group).  New or worsening symptoms were classified as adverse events.  It was recommended that medical treatment remain stable and adjustments necessitated by the individual’s psychiatric condition were recorded.  Medication was held constant during the 10-month protocol, except for transient increase in benzodiazepine therapy in 3 individuals and augmentation of neuroleptic treatment in 1 individual for exacerbated anxiety.  The primary outcome measure was severity of OCD as assessed by the Yale-Brown Obsessive Compulsive Scale (Y-BOCS) measured at the end of each period.  The Y-BOCS score was significantly lower at the end of active stimulation than at the end of the sham stimulation (mean score, 19 ± 8 vs. 28 ± 7; p=0.01) independent of the group and the period.  No significant carryover effect between treatment phases was detected.  Individuals who had active stimulation first (on-off group) tended to have a larger treatment effect than the off-on group (p=0.06).  Outcomes on secondary measures of global health and functioning were significantly better at the end of the stimulation period.  Scores on Montgomery and Asberg Depression Scale (MADRS), Brief Scale for Anxiety, neuropsychological ratings, and self-reported disability (Sheehan Disability Scale) did not differ significantly at the end of treatment and sham sessions.  Fifteen serious adverse events were reported in 11 individuals, the most serious a parenchymal brain hemorrhage.  Transient motor and psychiatric symptoms induced by active stimulation resolved spontaneously or with adjustment of stimulation settings.  Seven behavioral adverse events were reported in 5 individuals during stimulation.  Hypomania was the main psychiatric serious adverse event; symptoms resolved with adjustment of stimulation settings.  The authors note that the multicenter design might be a limitation of the study because of variation in targeting of stimulation.  In addition, in order to preserve blinding, stimulation settings were kept below the threshold known to induce adverse effects and may have been too low to reduce symptoms.  The authors concluded that these preliminary findings suggest that stimulation of the subthalamic nucleus may reduce the symptoms of severe forms of OCD but it is associated with a substantial risk of serious adverse events.

Denys et al. (2010) published the results of an RCT involving 16 subjects with OCD with a score greater than 28 on the Yale-Brown Obsessive Compulsive Scale (Y-BOCS) and failure of at least two selective serotonin reuptake inhibitor (SSRI) drugs.  Subjects underwent implantation of a DBS device to the nucleus accumbens and then entered an open 8-month treatment phase, followed by a double-blind crossover phase with randomly assigned 2-week periods of active or sham stimulation, ending with an open 12-month maintenance phase.  The authors reported that in the open phase, the mean Y-BOCS score decreased by 46%, from 33.7 (3.6) at baseline to 18.0 (11.4) after 8 months (p<0.001).  Of the total 16 subjects, 9 were noted to be responders, with a mean Y-BOCS score decrease of 23.7 (72%).  In the double-blind, sham-controlled phase in which data for 14 subjects was available, the mean Y-BOCS score difference between active and sham stimulation was 8.3 (25%; p=0.004).  Depression and anxiety decreased significantly.  Except for mild forgetfulness and word-finding problems, no permanent adverse events were reported.

The results of a third and largest report were published by Greenberg and colleagues in 2010.  In this multi-center case series study, DBS was implanted into the ventral anterior limb of the internal capsule and adjacent ventral striatum (VC/VS) in 26 subjects with severe treatment resistant OCD.  Subjects had a score of greater than or equal to 28 on the Y-BOCS, had failed a minimum of two trials with SSRIs, and had symptoms for a minimum of 5 years.  At the final 36 months post-implant time point, mean Y-BOCS reached 20.9 ± 2.4, down from 34.0 ± 0.5 (p=0.002), and the number of subjects meeting criteria for full response (decrease in Y-BOCS ≥ 35) was 61.5% (16/26).  A total of 73% of subjects had at least a 25% decrease in Y-BOCS.  Global assessment of functioning (GAS) score was available for 21 subjects, and rose from a mean of 34.8 ± 1.1 at baseline to 59.05 ± 3.3 at 36 months (p=0.006).  Serious adverse events included intracerebral hemorrhage in 2 subjects (7.7%) following lead insertion, and both cases resolved spontaneously.  Another subject (3.8%) developed generalized tonic-clonic seizures following implantation, necessitating treatment with phenytoin for 1 month postoperatively.  Seizures did not recur following cessation of medical therapy.  Finally, 1 subject (3.8%) developed a superficial wound infection which resolved successfully with medical treatment.  Lead replacement was required in 2 (7.7%) subjects due to breakage.  Therapy-related complications included 4 cases of increased depression or suicidal ideation in 3 subjects (11.5%), increased severity of OCD was reported in 3 subjects (11.5%), hypomania in 1 subject (3.8%), and domestic problems/irritability associated with stimulation were reported in 1 subject (3.8%).  The authors concluded that their results suggest that neural networks relevant to therapeutic improvement might be modulated more effectively at a more posterior target.

In 2015, Alonso and others conducted a meta-analysis of studies addressing the use of DBS for the treatment of OCD.  They included 31 studies involving 116 subjects in the analysis.  The studies mentioned above (Denys, 2010; Greenberg, 2010; Mallet, 2008) were included, and represented 50% of the subject population.  The remaining 28 studies accounted for the rest of the subject pool, representing a mean of 2.07 subjects per study (range=1-10).  Implantation in striatal areas, anterior limb of the internal capsule, ventral capsule and ventral striatum, nucleus accumbens and ventral caudate was reported in 83 subjects.  Implantation in the subthalamic nucleus was reported in 27 subjects, and implantation in the inferior thalamic peduncle was reported in 6 subjects.  Global percentage of Y-BOCS reduction was estimated at 45.1% and global percentage of responders at 60.0%.  The authors reported that better response was associated with older age at OCD onset and presence of sexual/religious obsessions and compulsions.  No significant differences were detected in efficacy between targets.  There were only 5 dropouts reported, and adverse effects were generally reported as mild, transient, and reversible.  The authors concluded that their analysis confirms that DBS constitutes a valid alternative to lesional surgery for individuals with severe, therapy-refractory OCD.  However, they noted that well-controlled, randomized studies with larger samples are needed to establish the optimal targeting and stimulation conditions and to extend the analysis of clinical predictors of outcome.

Deep Brain Stimulation for Epilepsy

Results from the large scale Stimulation of the Anterior Nuclei of Thalamus for Epilepsy (SANTE) trial, a double-blind RCT of DBS for epilepsy, were reported by Fisher (2010).  This study used a standard DBS device (Medtronic Mode 3387) stimulating the anterior nuclei.  All subjects underwent DBS implantation followed by 3 months of randomized and blinded active stimulation (n=54) or no stimulation (n=55).  This period was then followed by 9 months of active stimulation for all subjects.  At the end of the trial period, 13 subjects (11.8%) were followed through 2 years.  A total of 110 subjects had DBS electrode implantation.  One subject in the active group was not included in the data analysis and no explanation for this was provided in the article.  Both the active and control groups demonstrated significant decreases in seizure activity through the blinded period.  However, the control group trended towards baseline levels at the end of the third month.  The active group had a sustained and significant decrease (p=0.0017).  A difference between groups was only seen in the third month, in favor of the stimulated group (p=0.0023).  Changes in additional outcome measures did not show significant differences between groups.  During the blinded phase, complex partial seizures improved more in the stimulated group vs. controls (p=0.041).  Additionally, seizure-related injuries occurred more in controls vs. stimulated subjects (26% vs. 7%; p=0.01).  No differences were noted in subjects with prior treatment of vagus nerve stimulation.  At completion of the blinded phase, 108 (98.1%) subjects entered the open-label phase.  The median seizure frequency percent change from baseline for subjects with at least 70 diary days prior to the visit was -41% (n=99) at 13 months and -56% (n=81) at 25 months.  The 50% responder rate at 13, 25, and 37 months was 43%, 54% and 67%.  Through 13 months, 808 adverse events were reported in 109 participants; 55 events were categorized as serious and 238 were considered device-related.  The most common device-related events were paresthesias (18.2%), implant site pain (10.9%), and implant site infection (9.1%).  Five deaths were reported in the follow-up period, including 3 from sudden unexplained death in epilepsy (SUDEP), 1 subject from unobserved drowning in a bathtub, and 1 suicide.  None of the deaths were judged device-related by center investigators.  During the blinded phase, the experimental group reported more adverse events relating to depression (8 vs. 1) and memory impairment (7 vs. 1).  Subjects in the stimulation group experienced fewer seizure-related injuries (7.4%) vs. the control group (25.5%, p=0.01).  The authors state that DBS of the anterior nuclei in this population was mostly palliative in nature, but 14 participants (12.7%) became seizure-free for at least 6 months.  Additionally, significant benefits were seen in some subjects who were not previously helped by multiple drugs, vagus nerve stimulation (VNS), or epilepsy surgery.  Finally, they conclude by stating, “Additional clinical experience may help to establish the best candidates and stimulation parameters, and to further refine the risk–benefit ratio of this treatment.”  This is especially true in light of the significantly increased rate of depression-related adverse events reported in the experimental group.  It must be noted that this study only followed 13 subjects past 13 months, mitigating the utility of the longer-term data presented. 

Salanova and others published the long-term follow-up results of the SANTE trial in 2015.  Beginning 13 months following device implantation, 105 subjects receiving active stimulation were followed for an additional 4 years.  The authors reported that for subjects with at least 70 diary entries recorded at 1 year (n=99), median change for seizure frequency from baseline decreased by 41% (p<0.001), and by 69% at 5 years (n=59; p<0.001).  For the same population, reduction in the most severe type of seizure was 39% at 1 year (p<0.001) and 75% at 5 years (p<0.001).  During the 5-year study, 17 of 109 subjects (16%) reported a 6-month seizure-free interval.  A 2-year seizure-free interval was reported for 6 of 109 subjects (5.5%).  Mean improvement in the Liverpool Seizure Severity Score (LSSS) was 13.4 at 1 year and 18.3 at 5 years (p<0.001 for both).  Similarly, results from the Quality of Life in Epilepsy-31 (QOLIE-31) tool improved from baseline by 5.0 points at 1 year and 6.1 points at 5 years (p<0.001 for both).  A change of 5 points on this measure is considered clinically significant, and was experienced by 46% and 48% of subjects at 1 and 5 years.  Overall, 39 of the 110 subjects (35.5%) experienced some device-related serious adverse events, which predominantly occurred within the first months of implantation.  The most common were impact site infection in 10% of subjects and leads not within target area (8.2%).  Depression was reported in 32.7% of subjects over 5 years, but only 3 were considered device-related.  Memory impairment was reported in 25.5% of subjects.  SUDEP was reported in 7 subjects over the 5-year study period, but none were considered device-related by the data monitoring committee.  This study demonstrates significant long-term benefit from DBS for individuals with epilepsy, however, this was a relatively small and unblinded study. Further data from larger blinded RCTs are warranted. 

In a meta-analysis of the impact of sham vs. placebo treatments in studies of DBS for epilepsy, the authors reported that both sham surgical and acupuncture procedures provided significantly more placebo effect than oral placebo (Meissner, 2013).  They commented that clinicians need to remember that a relevant part of the overall effect they observe in practice might be due to nonspecific effects.  This is apparently true for these studies in the short-term.  However, in the Fisher study, the placebo/sham effect mostly disappeared by the end of the 3 month blinded phase in the control groups.

Deep Brain Stimulation for Other Conditions

Tye and colleagues (2009) investigated the effectiveness of DBS in treatment-resistant depression, OCD, and Tourette syndrome (TS).  The authors found that DBS treatment for TS was largely dependent upon electrode placement.  One small study (n=5) found that bilateral thalamic electrode placement reduces tic frequency and severity in refractory TS (Maciunas, 2007).  Other DBS electrode implantation targets for TS include the centromedian thalamic region (Okun, 2012; Servello, 2008) and the globus pallidus (Diederich, 2005).

In 2009, Porta reported the findings of a case series study involving 18 subjects who underwent bilateral thalamic DBS for TS.  At the 24 month follow-up point, there was a marked reduction in tic severity (p=0.001), improvement in obsessive-compulsive symptoms (p=0.009), anxiety symptoms (p=0.001), depressive symptoms (p=0.001), and subjective perception of social functioning/quality of life (p=0.002) in 15 of 18 subjects.  There were no substantial differences on measures of cognitive functions before and after DBS.  The authors concluded by stating, “Controlled studies on larger cohorts with blinded protocols are needed to verify that this procedure is effective and safe for selected patients with TS.”

Ackermans and colleagues (2011) noted that since 1999, 10 different brain areas have been described as a target for deep brain stimulation in Tourette syndrome.  They conducted a study of 8 individuals in a double-blind, randomized cross-over trial using reduction of tic severity as the primary outcome.  After surgery, the subjects were randomly assigned to 3 months stimulation followed by 3 months OFF stimulation (Group A) or vice versa (Group B).  The cross-over period was followed by 6 months ON stimulation.  Tic severity during ON stimulation was significantly lower than during OFF stimulation, with substantial improvement (37%) on the Yale Global Tic Severity Scale (mean 41.1 ± 5.4 versus 25.6 ± 12.8; p=0.046).  The authors concluded that these preliminary findings suggest efficacy of DBS for tic symptoms in TS; however, further RCTs on other targets are urgently needed since the optimal DBS target for TS is still unknown.

Welter (2017) published the results of a double-blind RCT involving 17 subjects with severe medically refractory Tourette syndrome who were treated with bilateral implantation of a deep brain stimulator with electrodes to the anterior globus pallidus.  Subjects were randomly assigned in a 1:1 fashion to either active (n=8) or sham stimulation (n=9).  At 2 months following implantation, all subjects had their stimulators activated to determine the study settings.  One month later subjects were randomized to their treatment group and followed in a blinded fashion for an additional 3 months.  After that period, all subjects had their devices activated in an open-label fashion for an additional 6 months.  Medication regimens were continued during the study.  A total of 16 subjects completed the double-blind study period.  The authors reported no significant differences between groups at 3 months with regard to Yale Global Tic Severity Scale (YGTSS) scores (p=1.0).  During the open-label phase, YGTSS scores improved significantly in all subjects (p=0.23).  Improvement of 25% or more in YGTSS scores from baseline to the end of the open-label phase was noted in 12 of the 16 completing subjects (p=0.0017).  At the end of the open-label phase the stimulators were turned off for 72 hours, at which time mean YGTSS scores decreased 75.7% (p=0.02).  During the blinded phase, no significant differences were noted between groups with regard to motor or vocal tic YGTSS subscales or any other measures.  Between baseline and the end of the open-label phase, significant differences in the motor and vocal tic YGTSS subscales, the Rush Video Rating Scale (RVRS), Global Assessment Functioning (GAF), the Asberg Depression Scale (MADRS), and Hospital Anxiety and Depression Scale (HADS) (anxiety), but not in Clinical Global Impression (CGI), the Brief Anxiety Scale (BAS), the HADS (depression), the Yale Brown Obsessive-Compulsive Scale (Y-BOCS), the Stroop interference index, Social Adjustment Scale Self- Reported (SAS-SR), or SF-36 scores.  A total of 15 serious adverse events were reported in 13 (68%) subjects, including infections leading to removal of the stimulator and electrodes in 4 (21%) subjects.  Transient headaches (n=5) and pain along the scars (n=2) were also reported.  Adverse events related to stimulation across both groups were reported in 17 subjects, including increased tic severity and anxiety, depressive symptoms, dysarthria, sleep disorder, and imbalance or abnormal movements resembling dyskinesia that resolved rapidly after stimulator adjustments.  The authors concluded that 3 months of DBS is insufficient to decrease tic severity for individuals with Tourette’s syndrome and further study was needed. Also in 2017, Martinez-Ramirez and colleagues reported an analysis of data from 185 subjects with Tourette syndrome included in the prospectively collected International Deep Brain Stimulation Database and Registry who were treated with bilateral DBS.  Surgical selection was made using local evaluations and recommendations, with no standardized inclusion or exclusion criteria.  Location of electrodes was likewise not standardized, with stimulation occurring at the centromedian thalamic region (n=93), anterior globus pallidus internus (n=41), posterior globus pallidus internus (n=25), and anterior limb of internal capsule (n=4).  The authors reported that the mean total YGTSS score improved from 75.01 at baseline to 41.19 at 1 year (p<0.001).  The mean motor tic subscore improved from 21.00 at baseline to 12.91 after 1 year (p<0.001), and the mean phonic tic subscore improved from 16.82 at baseline to 9.63 at 1 year (p<0.001).  The adverse event rate was 35.4% (56 of 158 subjects), with reports of intracranial hemorrhage (n=2), infection (n=5), and lead explantation (n=1).  The most common stimulation-induced adverse effects were dysarthria (n=10) and paresthesia (n=13).  The authors concluded that DBS was associated with symptomatic improvement in individuals with Tourette syndrome, but also with important adverse events.

The European Clinical Guidelines for Tourette Syndrome and Other Tic Disorders. Part IV: Deep Brain Stimulation (2011) stated that:

…Of the 63 patients reported so far in the literature 59 had a beneficial outcome following DBS with moderate to marked tic improvement. However, randomized controlled studies including a larger number of patients are still lacking. At present time, DBS in TS is still in its infancy.  
…However, among the European Society for the Study of Tourette Syndrome (ESSTS) working group on DBS in TS, there is general agreement that, at present time, DBS should only be used in adult, treatment resistant, and severely affected patients. It is highly recommended to perform DBS in the context of controlled trials.

Additional uses for DBS are being investigated.  For example, in psychosurgery there has been a shift of interest away from ablative techniques and toward deep brain stimulation.  However, studies of DBS for depression, obsessive-compulsive disorder, and anorexia are few and involve small numbers of subjects (Bergfeld, 2016; Lipsman, 2013, Sachdev, 2009; Schlaepfer, 2013; Wu, 2013).  Patel and colleagues (2011) reported a case study using DBS for treatment of severe, refractory hypertension.  In 2011, the National Institute for Health and Clinical Excellence (NICE) published DBS assessments in the treatment of trigeminal neuralgia and chronic pain syndromes.  They found that the available evidence at this time does not support this use.

Deep brain stimulation is also being studied as a treatment for tremors from other causes including, but not limited to, multiple sclerosis (MS), trauma and degenerative disorders.  In addition, DBS is being investigated to determine if functional improvement is achieved and maintained for other conditions such as chronic cluster headache, epilepsy and Tourette syndrome (TS). 

Cortical Stimulation

The RNS® System (NeuroPace, Mountain View, CA) consists of a cranially implanted, programmable cortical neurostimulator that senses and records brain activity through electrode-containing leads that are placed at the seizure focus.  The device provides what the manufacturer refers to as “responsive cortical stimulation,” which senses and records seizure activity and responds according to a pre-set program.  The system is intended to reduce the frequency of seizures in individuals with epilepsy.  On November 14, 2013, the RNS System was approved through the PMA (pre-market approval) process by the FDA with the following indication:

…as an adjunctive therapy in reducing the frequency of seizures in individuals 18 years of age or older with partial onset seizures who have undergone diagnostic testing that localized no more than 2 epileptogenic foci, are refractory to two or more antiepileptic medications, and currently have frequent and disabling seizures (motor partial seizures, complex partial seizures and/ or secondarily generalized seizures). The RNS® System has demonstrated safety and effectiveness in patients who average 3 or more disabling seizures per month over the three most recent months (with no month with fewer than two seizures), and has not been evaluated in patients with less frequent seizures.

The RNS system was approved on the basis of data from three trials; an initial Feasibility study, the Pivotal Trial, and a Long Term Treatment Investigation trial (LTT).  In the Feasibility study, the initial 4 subjects were involved in an open-label protocol.  The subsequent 61 subjects were enrolled in a double-blind RCT in which subjects received active or sham treatments.  The results of this study have been presented in conjunction with the results of the other two studies in the Summary of Safety and Effectiveness data presented to the FDA, but have not been reported separately in a peer-reviewed published paper.  As a result, no conclusions can be drawn based on the results of this study alone.

The Pivotal Trial results were reported by Morrell (2011).  This study involved the use of the RNS system in a double-blind RCT that initially enrolled 240 subjects.  A total of 49 subjects were excluded prior to implantation, leaving 191 subjects for analysis.  Inclusion criteria were 18-70 years of age, partial onset seizures refractory to at least two trials of anti-epileptic drugs, had experienced at least three disabling seizures per month and had either one or two epileptogentic regions localized.  All subjects underwent implantation of the RNS system followed by a 1-month break-in period followed by randomization.  Subjects were assigned to either active or sham therapy, and followed for the 12-week blinded treatment phase, then an 84-week open-label period where all subjects received active therapy.  The authors reported that the blind was successfully maintained (blinding index 0.5727).  Both groups experienced a reduction in mean seizure frequency during the first post-implant month prior to randomization.  However, this reduction abated during the blinded period in the sham group until, in the final month of the blinded period, seizure frequency approached pre-implant levels.  Mean seizure frequency was significantly reduced in the treatment group vs. the sham group (p=0.012) during the blinded period.  The responder rate (percentage of subjects with a ≥ 50% reduction in seizures) over the blinded period was not significant overall, with 29% in the treatment group responding vs. 27% in the sham group.  However, seizure-free days over the first month continued to increase in the treatment group but declined for the sham group.  By the third month, the treatment group had 27% fewer days with seizures vs. 16% fewer days in the sham group (p=0.048).  During the open-label period, the sham group demonstrated a statistically significant reduction in mean seizure frequency compared to the pre-implant period (p=0.04).  Across all subjects, the seizure reduction was sustained, and even improved, over time.  The responder rate at 1 year post-implant was 43% (n=177) and 46% (n=102) at 2 years.  As of the data cutoff date, 13 subjects (7.1%) were seizure-free over the most recent 3-month period.  The Quality of Life in Epilepsy-89 (QOLIE-89) assessment tool overall t scores were significantly improved in both groups at the end of the blinded period (p=0.040), 1 year (p<0.001) and 2 years (p=0.016).  During the blinded period, there was no difference between the treatment and sham groups in the frequency of cognitive adverse events, or any neuropsychological measure through 2 years.  No adverse changes in mood inventories were reported at any time point in the study.  The serious adverse event rate for medical and surgical events for the first 84 weeks was 18.3%.  This compares favorably to comparator rates for DBS.  There was no difference between the treatment and sham groups in the percentage of subjects with mild or serious adverse events over the blinded period, and included intracranial hemorrhage due to surgical complications and subdural hematomas attributed to seizure-related head trauma.  Six subjects died, but none were attributed to responsive cortical stimulation treatment. The authors conclude with:

Improvements in QOL overall and in domains related to health concerns, social functioning, and cognition support the clinical meaningfulness of the treatment response. Safety was acceptable compared to alternative and comparable procedures and to the risks of frequent seizures. Stimulation was well-tolerated and there were no adverse effects on cognition or mood. Given these findings, responsive cortical stimulation may provide another much-needed treatment option for persons with medically intractable partial seizures.

The final part of the FDA submission data came from the LTT, which was composed of 57 subjects who completed the Feasibility trial and another 173 subjects who completed the Pivotal Trial.  These subjects are being followed for a maximum of 7 years, and the study is ongoing. 

The first published report of data from the LTT was made available in 2015 (Bergey, 2015).  This report included data from 191 subjects who have completed data at the 6-year cutoff point, but data are presented based on the entire subject pool of 256.  The median reduction in seizures was 60% at 3 years and 66% at 6 years.  The responder rates at the same time points were 58% and 59%.  Adjusted response rates taking into account withdrawals at the same time points were 58% and 56%.  Based on data from the last 3 months of the collection period, 84% of subjects had some improvement, 60% had 50% or greater reduction in seizure frequency, and 16% were seizure free.  Only 8% had a 50% or greater increase in seizure activity.  Over one-third of subjects experienced a 3-month seizure-free interval, and 23% experienced one of 6 months or longer.  QOLIE-89 measures through year 5 continued to improve significantly (p<0.001).  Serious events were reported in 2.5% or more of the subjects at any time during the study period.  Three intracranial hemorrhages were reported at 18 months, 2.5 years, and 2.8 years following device implantation.  Death was reported for 11 subjects, including 2 suicides, 1 status epilepticus, 1 lymphoma, and 7 possible SUDEP.  The device was off at the time of 2 SUDEP deaths.  These results are promising, and demonstrate continued significant benefit to the use of the RNS system in subjects with epilepsy.

In 2014, Heck and colleagues published the final 2-year results of the Pivotal trial.  The percent change in seizures at the end of the blinded period was -37.9% in the active and -17.3% in the sham group (p=0.012).  The median percent reduction in seizures in the open-label period was 44% at 1 year and 53% at 2 years, which represents a progressive and significant improvement with time (p<0.0001).  The authors reported no differences between groups with regard to the rate of serious adverse events, which were consistent with the known risks of an implanted medical device, seizures, and of other epilepsy treatments.  There were no adverse effects on neuropsychological function or mood.

Use of the RNS system for the treatment of mesial temporal lobe (MTL) epilepsy was evaluated in a retrospective study involving data from 111 subjects who were involved in the Feasibility (n=16), Pivotal (n=95) studies, and LTT studies (92 Pivotal subjects continued into the LTT) (Geller, 2017).  The mean follow-up at the time of data cutoff was 6.1 ± 2.2 years.  Subjects had one to four leads placed during the initial procedure, with one lead (n=1), two leads (n=92), three leads (n=4) or four leads placed (n=14).  Only depth leads were placed in 76 subjects, 29 had both depth and strip leads, and 6 had only strip leads.  The authors reported that disabling seizures were reduced by a median 66.5% at 6 years, and the 50% responder rate reached 64.6%.  No seizures were reported by 20.8% of subjects in the last 3 months.  Over the entire open-label period, 45% of subjects reported seizure-free intervals lasting ≥ 3 months, 29% lasting ≥ 6 months, and 15% lasting ≥ 1 year.  There was no difference in seizure reduction in subjects with and without mesial temporal sclerosis (MTS, p=0.42), bilateral MTL onsets (p=0.97), prior resection (p=0.54), prior intracranial monitoring, and prior VNS (p=0.78).  There were only two device-related (related or uncertain as categorized by the investigator) serious adverse events reported in 13 of subjects, including superficial soft tissue implant-site infection and device lead damage.  Implant-site skin erosion was reported in 2 subjects.  Lead replacement due to lead damage occurred in 7 subjects.  Three subjects had a serious AE related to intracranial hemorrhage, and two were categorized as device related.  A total of 6 deaths were reported, 1 suicide and 5 attributed to possible (n=2), probable (n=1), or definite (n=2) SUDEP.  Other adverse events reported include photopsia (n=16), memory impairment (n=7), and depression (n=2).

A similar study was described by Jobst in 2017 involving 126 subjects from the Pivotal (n=45) and LTT studies (n=81) with frontal-onset seizures and leads in either Broca’s or Wernicke’s areas were included.  The mean follow-up at the time of data cutoff was 6.1 ± 2.6 years.  Seizure data were available for 120 subjects with > 1 year of follow-up, 87 had at least 6 years of follow-up.  The reported median percent reduction in seizures was 44% after 2 years, 61% after 5 years, and 76% after 6 years.  During the open-label period, 37% of subjects had at least one seizure-free interval lasting ≥ 3 months, 26% had at least one lasting ≥ 6 months, and 14% had at least one lasting ≥ 1 year.  Previous surgery or prior VNS had no impact on seizure reduction results (p=0.12 and p=0.20, respectively).  Both subjects with and without structural lesions experienced a reduction in seizure frequency, and the reduction was greater in subjects with a structural lesion than in those without, and the difference between these groups was significant over the entire follow-up (p=0.02).  Serious adverse events related to intracranial hemorrhage were reported in 9 subjects, with 6 attributed to seizure-related head trauma.  Two subjects had cerebral hemorrhages several years after implantation, with one considered device related.  Serious infection-related adverse events were reported in 13 subjects, with 9 resulting in explantation of the stimulator and 6 in explantation of the leads as well.  Two subjects developed scalp erosions over the neurostimulator.  Death was reported for 5 subjects with 1 suicide, 1 due to status epilepticus, 1 due to lymphoma, and 2 attributed to definite SUDEP.

Devinsky (2018) reported the results of a retrospective study investigating the incidence of SUDEP in 707 subjects implanted with the RNS system.  The authors reported that there were 14 all-cause total deaths in the study cohort with 2208 years of post-implantation follow-up data.  There were two possible, one probable and four definite SUDEP events, resulting in an overall, standardized mortality ratio (SMR) for probable and definite SUDEP of 0.75 (95%, confidence interval [CI], 0.27-1.65).  Two subjects who suffered SUDEP did not have their stimulator devices activated at the time of death.  The authors reported a SUDEP rate of 2.0/1000 patient stimulation years, and state that this is, “is favorable relative to treatment-resistant epilepsy patients randomized to the placebo arm of add-on drug studies or with seizures after resective surgery.”

Responsive cortical stimulation with the RNS system has been demonstrated to be safe and effective in select individuals with partial onset seizures who have undergone diagnostic testing that localized no more than two epileptogenic foci, are refractory to two or more antiepileptic medications, and are currently having an average of three or more disabling seizures per month.  Studies have shown significant improvements in seizure frequency.

Cerebellar Stimulation/Pacing

Cerebellar stimulation/pacing is electrical stimulation using surgically implanted electrodes on the surface of the cerebellum and has been proposed as one way to treat some neurological disorders.  At this time, there is inadequate information available to make an assessment of the clinical usefulness of this procedure.

Background/Overview

Various forms of electrical stimulation have been investigated as an alternative to permanent neuroablative procedures, such as thalamotomy and pallidotomy for neuroelectrical conditions.  The technique using deep brain stimulation (DBS) has been most thoroughly investigated as an alternative to thalamotomy for unilateral control of essential tremor, and tremor associated with Parkinson's disease (PD).  DBS has also been investigated in individuals with primary dystonia, defined as a neurological movement disorder characterized by involuntary muscle contractions, which force certain parts of the body into abnormal, contorted and painful movements or postures and which is unrelated to any other neurological condition.  Treatment options for dystonia include oral or injectable medications (i.e., botulinum toxin) and destructive surgical or neurosurgical interventions (i.e., thalamotomies or pallidotomies) when conservative therapies fail.

Deep brain stimulation involves the stereotactic placement of an electrode into the brain (i.e., thalamus, globus pallidus, or subthalamic nucleus).  The electrode is initially attached to a temporary transcutaneous cable for short-term stimulation to validate treatment effectiveness.  Several days later, the individual returns to surgery for permanent subcutaneous implantation of the cable and a radiofrequency-coupled or battery-powered programmable stimulator.  The electrode is typically implanted unilaterally on the side corresponding to the most severe symptoms.  However, the use of bilateral stimulation using two electrode arrays has also been investigated in individuals with bilateral, severe symptoms.

After implantation, noninvasive programming of the neurostimulator can be adjusted to the individual's symptoms.  This feature may be important for individuals with PD, whose disease may progress over time, requiring different neurostimulation parameters.  Setting the optimal neurostimulation parameters may involve the balance between optimal symptom control and appearance of side effects of neurostimulation, such as dysarthria, disequilibrium, or involuntary movements.

Cortical stimulation is a newer technology proposed for the treatment of epilepsy.  Currently the only device available for this type of treatment is the RNS System (NeuroPace, Inc., Mountain View, CA).  The RNS System involves implantation of electrodes onto the surface the brain near areas associated with seizure activity.  Those electrodes are then attached to a control/generator unit which is also implanted in the head.  The control unit monitors and records electrical activity of the brain and provides electrical stimulation when needed.  Following a trial period, the initial brain activity record is evaluated by a doctor.  The record is used to identify the individual’s unique pre-seizure electrical brain activity patterns and to set the RNS device to recognize and react to those patterns.  Once the recognition parameters are set, the device monitors brain activity for the pre-set patterns of electrical activity.  If those patterns are detected the device activates to provide stimulation through the electrodes with the goal of preventing a seizure.

Cerebellar stimulation or pacing is a similar technique to DBS, but works in the cerebellar portion of the brain.  At this time there is little information about the use of this technology in humans.

Definitions

Cerebellar stimulation/pacing: A proposed treatment of neurological disorders that involves electrical stimulation of the cerebellum part of the brain.

Dystonia: Covers a diverse group of movement disorders, all of which are characterized by involuntary muscle contractions that may cause twisting and repetitive movements or abnormal postures; dystonia is the most severe form of a group of movement disorders called dyskinesias.

Essential tremor (ET): A chronic, incurable condition with unknown cause characterized by involuntary, rhythmic tremor of a body part, most typically the hands and arms.

Globus pallidus interna (GPi): A part of the brain involved with movement.

Humanitarian Device Exemption (HDE): Similar to a premarket approval (PMA) application, but is exempt from the effectiveness requirements of a PMA.  An HDE application is not required to contain the results of scientifically valid clinical investigations demonstrating that the device is effective for its intended purpose and does not pose an unreasonable or significant risk of illness or injury.  The use of the device is limited to 4000 or less individuals per year.

Multiple sclerosis: A condition of the nervous system that results in a wide variety of symptoms.

Parkinson's disease: A progressive, incurable disease caused by the slow continuous loss of nerve cells in the part of the brain that controls muscle movement.

Post-traumatic dyskinesia: A condition where movement is altered or absent due to a traumatic injury.

Primary dystonia: A type of dystonia which is not due to a secondary cause such as stroke, cerebral palsy, tumor, trauma, infection, multiple sclerosis, medications, or a neurodegenerative disease.

Secondary dystonia: A type of dystonia which is associated with a known, acquired cause or additional neurologic abnormality where symptoms of involuntary muscle contractions are related to other conditions such as stroke, trauma, toxic substance exposure or asphyxia.

Subthalamic nucleus (STN): A part of the brain involved with movement.

Tourette's syndrome or Tourette syndrome (TS): A neurological disorder characterized by multiple facial and other body tics, usually beginning in childhood or adolescence and often accompanied by grunts and compulsive utterances, such as interjections and obscenities: TS is also called Gilles de la Tourette syndrome.

Unified Parkinson's Disease Rating Scale (UPDRS): UPDRS is a rating tool to follow the longitudinal course of Parkinson's Disease and is made up of three sections: 1) mentation, behavior and mood, 2) activities of daily living and 3) motor sections evaluated by interview.

Ventralis intermediate nucleus of the thalamus (Vim): A part of the brain involved with movement.

Coding

The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

Deep Brain Stimulation
When services may be Medically Necessary when criteria are met:

CPT

 

61863

Twist drill, burr hole, craniotomy, or craniectomy with stereotactic implantation of neurostimulator electrode array in subcortical site (eg, thalamus, globus pallidus, subthalamic nucleus, periventricular, periaqueductal gray), without use of intraoperative microelectrode recording; first array

61864

Twist drill, burr hole, craniotomy, or craniectomy with stereotactic implantation of neurostimulator electrode array in subcortical site (eg, thalamus, globus pallidus, subthalamic nucleus, periventricular, periaqueductal gray), without use of intraoperative microelectrode recording; each additional array

61867

Twist drill, burr hole, craniotomy, or craniectomy with stereotactic implantation of neurostimulator electrode array in subcortical site (eg, thalamus, globus pallidus, subthalamic nucleus, periventricular, periaqueductal gray), with use of intraoperative microelectrode recording; first array

61868

Twist drill, burr hole, craniotomy, or craniectomy with stereotactic implantation of neurostimulator electrode array in subcortical site (eg, thalamus, globus pallidus, subthalamic nucleus, periventricular, periaqueductal gray), with use of intraoperative microelectrode recording; each additional array

61885

Insertion or replacement of cranial neurostimulator pulse generator or receiver, direct or inductive coupling; with connection to a single electrode array

61886

Incision and subcutaneous placement of cranial neurostimulator pulse generator or receiver, direct or inductive coupling; with connection to 2 or more electrode arrays

 

 

HCPCS

 

C1767

Generator; neurostimulator (implantable), nonrechargeable

C1820

Generator; neurostimulator (implantable), non high-frequency with rechargeable battery and charging system

C1822

Generator, neurostimulator (implantable), high frequency, with rechargeable battery and charging system

L8679

Implantable neurostimulator, pulse generator, any type

L8680

Implantable neurostimulator electrode, each

L8682

Implantable neurostimulator radiofrequency receiver

L8683

Radiofrequency transmitter (external) for use with implantable neurostimulator radiofrequency receiver

L8685

Implantable neurostimulator pulse generator, single array, rechargeable, includes extension

L8686

Implantable neurostimulator pulse generator, single array, non-rechargeable, includes extension

L8687

Implantable neurostimulator pulse generator, dual array, rechargeable, includes extension

L8688

Implantable neurostimulator pulse generator, dual array, non-rechargeable, includes extension

 

 

ICD-10 Procedure

 

 

For the following codes when specified as deep brain stimulator leads:

00H00MZ-00H04MZ

Insertion of neurostimulator lead into brain [by approach; includes codes 00H00MZ, 00H03MZ, 00H04MZ]

00H60MZ-00H64MZ

Insertion of neurostimulator lead into cerebral ventricle [by approach; includes codes 00H60MZ, 00H63MZ, 00H64MZ]

 

 

ICD-10 Diagnosis

 

G20

Parkinson's disease

G21.0-G21.9

Secondary parkinsonism

G24.1

Genetic torsion dystonia

G24.2

Idiopathic nonfamilial dystonia

G24.3

Spasmodic torticollis

G24.8

Other dystonia

G24.9

Dystonia, unspecified

G25.0

Essential tremor

When services are Investigational and Not Medically Necessary:
For the procedure and diagnosis codes listed above when criteria are not met, for deep brain stimulation for all other diagnoses not listed; or when the code describes a procedure indicated in the Position Statement section as investigational and not medically necessary.

Cortical Stimulation
When services may be Medically Necessary when criteria are met:

CPT

 

61850

Twist drill or burr hole(s) for implantation of neurostimulator electrodes, cortical

61860

Craniectomy or craniotomy for implantation of neurostimulator electrodes, cerebral, cortical

64999

Unlisted procedure, nervous system [when specified as implantation of cortical neurostimulator]

 

 

HCPCS

 

C1767

Generator; neurostimulator (implantable), nonrechargeable

L8679

Implantable neurostimulator, pulse generator, any type

L8680

Implantable neurostimulator electrode, each

 

 

ICD-10 Procedure

 

 

For the following codes when specified as cortical stimulator leads:

00H00MZ-00H04MZ

Insertion of neurostimulator lead into brain [by approach; includes codes 00H00MZ, 00H03MZ, 00H04MZ]

00H60MZ-00H64MZ

Insertion of neurostimulator lead into cerebral ventricle [by approach; includes codes 00H60MZ, 00H63MZ, 00H64MZ]

 

 

ICD-10 Diagnosis

 

G40.001-G40.919

Epilepsy and recurrent seizures

When services are Investigational and Not Medically Necessary:
For the procedure and diagnosis codes listed above when criteria are not met, for cortical stimulation for all other diagnoses not listed; or when the code describes a procedure indicated in the Position Statement section as investigational and not medically necessary.

Cerebellar Stimulation
When Services are also Investigational and Not Medically Necessary:

CPT

 

61870

Craniectomy or craniotomy for implantation of neurostimulator electrodes, cerebellar, cortical

 

 

ICD-10 Diagnosis

 

 

All diagnoses

References

Peer Reviewed Publications:

  1. Ackermans L, Duits A, van der Linden C, et al. Double-blind clinical trial of thalamic stimulation in patients with Tourette syndrome. Brain. 2011; 134(Pt 3):832-844.
  2. Alonso P, Cuadras D, Gabriëls L, et al. Deep brain stimulation for obsessive-compulsive disorder: a meta-analysis of treatment outcome and predictors of response. PLoS One. 2015; 10(7):e0133591.
  3. Anderson WS, Kossoff EH, Bergey GK, Jallo GI. Implantation of a responsive neurostimulator device in patients with refractory epilepsy. Neurosurg Focus. 2008; 25(3):E12.
  4. Bergey GK, Morrell MJ, Mizrahi EM, et al. Long-term treatment with responsive brain stimulation in adults with refractory partial seizures. Neurology. 2015; 84(8):810-817.
  5. Bergfeld IO, Mantione M, Hoogendoorn ML, et al. Deep brain stimulation of the ventral anterior limb of the internal capsule for treatment-resistant depression: a randomized clinical trial. JAMA Psychiatry. 2016; 73(5):456-464.
  6. Cif L, El Fertit H, Vayssiere N, et al. Treatment of dystonic syndromes by chronic electrical stimulation of the internal globus pallidus. J Neurosurg Sci. 2003; 47(1):52-55.
  7. Denys D, Mantione M, Figee M, et al. Deep brain stimulation of the nucleus accumbens for treatment-refractory obsessive-compulsive disorder. Arch Gen Psychiatry. 2010; 67(10):1061-1068.
  8. Deuschl, G, Wenzelburger R, Loffler K, et al. Essential tremor and cerebellar dysfunction clinical and kinematic analysis of intention tremor. Brain. 2000; 123(Pt 8):1568-1580.
  9. Devinsky O, Friedman D, Duckrow RB, et al. Sudden unexpected death in epilepsy in patients treated with brain-responsive neurostimulation. Epilepsia. 2018; 59(3):555-561.
  10. Diederich NJ, Kalteis K, Stamenkovic M, et al. Efficient internal pallidal stimulation in Gilles de la Tourette syndrome: a case report. Mov Disord. 2005; 20(11):1496-1499.
  11. Fields JA, Troster AI, Woods SP, et al. Neuropsychological and quality of life outcomes 12 months after unilateral thalamic stimulation for essential tremor. J Neurol Neurosurg Psychiatry. 2003; 74(3):305-311.
  12. Figuerias-Mendez R, Regidor I, Riva-Meana C, Magarinos-Ascone CM. Further supporting evidence of beneficial subthalamic stimulation in Parkinson’s patients. Neurology. 2002; 58(3):469-470.
  13. Fisher R, Salanova V, Witt T, et al.; SANTE Study Group. Electrical stimulation of the anterior nucleus of thalamus for treatment of refractory epilepsy. Epilepsia. 2010; 51(5):899-908.
  14. Fraix V, Houeto JL, Lagrange C, et al. Clinical and economic results of bilateral subthalamic nucleus stimulation in Parkinson’s disease. J Neurol Neurosurg Psychiatry. 2006; 77(4):443-449.
  15. Geller EB, Skarpaas TL, Gross RE, et al. Brain-responsive neurostimulation in patients with medically intractable mesial temporal lobe epilepsy. Epilepsia. 2017; 58(6):994-1004.
  16. Goetz CG, Poewe W, Rascol O, et al. Movement Disorder Society Task Force report on the Hoehn and Yahr staging scale: status and recommendations. Mov Disord. 2004; 19(9):1020-1028.
  17. Goetz CG, Poewe W, Rascol O, Sampaio C. Evidence-based medical review update: pharmacological and surgical treatments of Parkinson’s disease: 2001 to 2004. Mov Disord. 2005; 20(5):532-539.
  18. Goetz CG, Tilley BC, Shaftman SR, et al. Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): scale presentation and clinimetric testing results. Mov Disord. 2008; 23(15):2129-2170. 
  19. Greenberg BD, Gabriels LA, Malone D, et al. Deep brain stimulation of the ventral internal capsule/ventral striatum for obsessive-compulsive disorder: worldwide experience. Mol Psychiatry. 2010; 15(1):64-79.
  20. Heck CN, King-Stephens D, Massey AD, et al. Two-year seizure reduction in adults with medically intractable partial onset epilepsy treated with responsive neurostimulation: final results of the RNS System Pivotal trial. Epilepsia. 2014; 55(3):432-441.
  21. Jobst BC, Kapur R, Barkley GL, et al. Brain-responsive neurostimulation in patients with medically intractable seizures arising from eloquent and other neocortical areas. Epilepsia. 2017; 58(6):1005-1014.
  22. Levine CB, Fahrbach KR, Siderowf AD, et al. Diagnosis and treatment of Parkinson's disease: a systematic review of the literature. Evid Rep Technol Assess (Summ). 2003; (57):1-4.
  23. Lipsman N, Woodside DB, Giacobbe P, et al. Subcallosal cingulate deep brain stimulation for treatment-refractory anorexia nervosa: a phase 1 pilot trial. Lancet. 2013; 381(9875):1361-1370.
  24. Maciunas RJ, Maddux BN, Riley DE, et al. Prospective randomized double-blind trial of bilateral thalamic deep brain stimulation in adults with Tourette syndrome. J Neurosurg. 2007; 107(5):1004-1014.
  25. Mallet L, Polosan M, Jaafari N, et al. Subthalamic nucleus stimulation in severe obsessive-compulsive disorder. N Engl J Med. 2008; 359(20):2121-2134.
  26. Martinez-Ramirez D, Jimenez-Shahed J, Leckman JF, et al. Efficacy and safety of deep brain stimulation in Tourette syndrome the international Tourette syndrome deep brain stimulation public database and registry. JAMA Neurol. 2018; 75(3):353-359.
  27. Meissner K, Fässler M, Rücker G, et al. Differential effectiveness of placebo treatments: a systematic review of migraine prophylaxis. JAMA Intern Med. 2013; 173(21):1941-1951.
  28. Merello M, Nouzeilles MI, Kuzis G, et al. Unilateral radiofrequency lesion versus electrostimulation of posteroventral pallidum: a prospective randomized comparison. Mov Disord. 1999; 14(1):50-56.
  29. Morrell MJ; RNS System in Epilepsy Study Group. Responsive cortical stimulation for the treatment of medically intractable partial epilepsy. Neurology. 2011; 77(13):1295-1304.
  30. Obeso JA, Guridi J, Rodriguez-Oroz MC, et al.; Deep Brain Stimulation for Parkinson’s Disease Study Group. Deep brain stimulation of the subthalamic nucleus or the pars interna of the globus pallidus in Parkinson’s Disease. N Engl J Med. 2001; 345(13):956-963.
  31. Okun MS, Foote KD, Wu SS, et al. A trial of scheduled deep brain stimulation for Tourette syndrome: moving away from continuous deep brain stimulation paradigms. JAMA Neurol. 2013; 70(1):85-94.
  32. Olanow CW, Watts RL, Koller WC. An algorithm (decision tree) for the management of Parkinson’s disease (2001): treatment guidelines. Neurology. 2001; 56(11 Suppl 5):S1-S88.
  33. Ondo W, Almaguer M, Jankovic J, Simpson RK. Thalamic deep brain stimulation: comparison between unilateral and bilateral placement. Arch Neurol. 2001; 58(2):218-222.
  34. Pahwa R, Lyons KL, Wilkinson SB, et al. Bilateral thalamic stimulation for the treatment of essential tremor. Neurology. 1999; 53(7):1447-1450.
  35. Patel N, Javed S, Khan S, et al. Deep brain stimulation relieves refractory hypertension. Neurology. 2011; 76(4):405-407.
  36. Piper M, Abrams GM, Marks WJ Jr. Deep brain stimulation for the treatment of Parkinson’s disease: overview and impact on gait and mobility. NeuroRehabilitation. 2005; 20(3):223-232.
  37. Porta M, Brambilla A, Cavanna AE, et al. Thalamic deep brain stimulation for treatment-refractory Tourette syndrome: two-year outcome. Neurology. 2009; 73(17):1375-1380.
  38. Portman AT, van Laar T, Staal MJ, et al. Chronic stimulation of the subthalamic nucleus increases daily on-time without dyskinesia in advanced Parkinson’s disease. Parkinsonism Relat Disord. 2006; 12(3):143-148.
  39. Rehncrona S, Johnels B, Widner H, et al. Long-term efficacy of thalamic deep brain stimulation for tremor: double-blind assessments. Mov Disord. 2003; 18(2):163-170.
  40. Roper JA, Kang N, Ben J, et al. Deep brain stimulation improves gait velocity in Parkinson's disease: a systematic review and meta-analysis. J Neurol. 2016; 263(6):1195-1203.
  41. Sachdev PS, Chen X. Neurosurgical treatment of mood disorders: traditional psychosurgery and the advent of deep brain stimulation. Curr Opin Psychiatry. 2009; 22(1):25-31.
  42. Salanova V, Witt T, Worth R, et al.; SANTE Study Group. Long-term efficacy and safety of thalamic stimulation for drug-resistant partial epilepsy. Neurology. 2015; 84(10):1017-1025.
  43. Schlaepfer TE, Bewernick BH, Kayser S, et al. Rapid effects of deep brain stimulation for treatment-resistant major depression. Biol Psychiatry. 2013; 73(12):1204-1212.
  44. Schuepbach WM, Rau J, Knudsen K, et al.; EARLYSTIM Study Group. Neurostimulation for Parkinson's disease with early motor complications. N Engl J Med. 2013; 368(7):610-622.
  45. Schuurman PR, Bosch DA, Bossuyt PM, et al. A comparison of continuous thalamic stimulation and thalamotomy for suppression of severe tremor. N Engl J Med. 2000; 342(7):461-468.
  46. 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(2):136-142.
  47. Tan ZG, Zhou Q, Huang T, Jiang Y. Efficacies of globus pallidus stimulation and subthalamic nucleus stimulation for advanced Parkinson's disease: a meta-analysis of randomized controlled trials. Clin Interv Aging. 2016; 11:777-786.
  48. Tye SJ, Frye MA, Lee KH. Disrupting disordered neurocircuitry: treating refractory psychiatric illness with neuromodulation. Mayo Clin Proc. 2009; 84(6):522-532.
  49. Vidailhet M, Vercueil L, Houeto JL, et al. Bilateral deep-brain stimulation of the globus pallidus in primary generalized dystonia. N Engl J Med. 2005; 352(5):459-467.
  50. Volkmann J, Mueller J, Deuschl G, et al.; DBS study group for dystonia. Pallidal neurostimulation in patients with medication-refractory cervical dystonia: a randomised, sham-controlled trial. Lancet Neurol. 2014; 13(9):875-884.
  51. Volkmann J, Wolters A, Kupsch A, et al.; DBS study group for dystonia. Pallidal deep brain stimulation in patients with primary generalised or segmental dystonia: 5-year follow-up of a randomised trial. Lancet Neurol. 2012; 11(12):1029-1038.
  52. Weaver F, Follett K, Hur K, et al. Deep brain stimulation in Parkinson disease: a metaanalysis of patient outcomes. J Neurosurg. 2005; 103(6):956-967.
  53. Welter ML, Houeto JL, Thobois S, et al.; STIC study group. Anterior pallidal deep brain stimulation for Tourette's syndrome: a randomised, double-blind, controlled trial. Lancet Neurol. 2017; 16(8):610-619.
  54. Welter ML, Mallet L, Houeto JL, et al. Internal pallidal and thalamic stimulation in patients with Tourette syndrome. Arch Neurol. 2008; 65(7):952-957. 

Government Agency, Medical Society, and Other Authoritative Publications:

  1. American Academy of Neurology. Evaluation of surgery for Parkinson's disease: a report of the Therapeutics & Technology Assessment Subcommittee of the Task Force on Surgery for Parkinson's Disease. Neurology. 1999; 53(9):1910-1921.
  2. Centers for Medicare and Medicaid Services. National Coverage Determination for Deep Brain Stimulation for Essential Tremor and Parkinson's Disease. NCD #160.24. Effective April 1, 2003. Available at: http://www.cms.gov/medicare-coverage-database/indexes/ncd-by-chapter-and-section-index.aspx?bc=BAAAAAAAAAAA&. Accessed on March 26, 2018.
  3. Müller-Vahl KR, Cath DC, Cavanna AE, et al. European clinical guidelines for Tourette syndrome and other tic disorders. Part IV: deep brain stimulation. Eur Child Adolesc Psychiatry. 2011; 20(4):209-217.
  4. Pahwa R, Factor AS, Lyons KE, et al. Practice parameter: treatment of Parkinson disease with motor fluctuations and dyskineais (an evidence-based review): report of the Quality Standards Subcommittee of the Anerican Academy of Neurology. Neurology. 2006; 66(7):983-995.
  5. Sprengers M, Vonck K, Carrette E, Marson AG, Boon P. Deep brain and cortical stimulation for epilepsy. Cochrane Database Syst Rev. 2017 Jul 18;7:CD008497.
  6. U.S. Food and Drug Administration Center for Devices and Radiological Health. Medtronic Activa® Parkinson's Control Therapy. Premarket Approval. 960009. Rockville, MD: FDA. January 14, 2002. Available at:  https://www.accessdata.fda.gov/scripts/cdrh/cfdocs/cfpma/pma.cfm?id=P960009S051. Accessed on March 26, 2018.
  7. U.S. Food and Drug Administration Center for Devices and Radiological Health. Humanitarian Device Approval. Medtronic Activa® Dystonia Therapy. H020007. Rockville, MD: FDA. April 15, 2003. Available at: https://www.accessdata.fda.gov/cdrh_docs/pdf2/H020007A.pdf. Accessed on March 26, 2018.
  8. U.S. Food and Drug Administration Center for Devices and Radiological Health. Humanitarian Device Approval. Medtronic Reclaim™ Deep Brain Stimulation for Obsessive Compulsive Disorder (OCD) Therapy H050003. Rockville, MD: FDA. February 4, 2009. Available at: http://www.accessdata.fda.gov/cdrh_docs/pdf5/H050003a.pdf. Accessed on March 26, 2018.
  9. U.S. Food and Drug Administration. NeuroPace® RNS® System. P100026- Summary of Safety and Effectiveness Data. Available at: https://www.accessdata.fda.gov/cdrh_docs/pdf10/P100026B.pdf. Accessed on March 26, 2018.
  10. U.S. Food and Drug Administration. RNS® System – P100026. Available at: https://www.accessdata.fda.gov/cdrh_docs/pdf10/P100026A.pdf. Accessed on March 26, 2018.
  11. Wu H, Van Dyck-Lippens PJ, Santegoeds R, et al. Deep-brain stimulation for anorexia nervosa. World Neurosurg. 2013; 80(3-4):S29.e1-10.
  12. Zesiewicz TA, Elble R, Louis ED, et al. Practice parameter: therapies for essential tremor: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2005; 64(12):2008-2020.
  13. Zesiewicz TA, Elble RJ, Louis RJ, et al. Evidence-based guideline update: Treatment of essential tremor report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology. 2011; 77(19):1752–1755.
  14. Zesiewicz TA, Sullivan KL, Arnulf I, et al. Practice parameter: treatment of nonmotor symptoms of Parkinson disease: report of the Quality Standards Subcommittee of the Anerican Academy of Neurology. Neurology. 2010; 74(11):924-931.
Websites for Additional Information
  1. American Association of Neurological Surgeons. Deep Brain Stimulation. 2007. Available at: http://www.aans.org/Patient%20Information/Conditions%20and%20Treatments/Deep%20Brain%20Stimulation.aspx. Accessed on April 26, 2018.
  2. National Institute of Neurological Disorders and Stroke (NINDS). National Institutes of Health (NIH). NINDS Disorders Information Page. Available at: https://www.ninds.nih.gov/Disorders/all-disorders. Accessed on April 26, 2018.
  3. National Library of Medicine. Medical Encyclopedia: Parkinson disease. Available at: http://www.nlm.nih.gov/medlineplus/ency/article/000755.htm. Accessed on April 26, 2018.
Index

Activa Tremor Control System
Brio Neurostimulation System
Cerebellar Stimulation/Pacemaker
Deep Brain Stimulation for Tremor
Essential Tremor
Parkinson Disease
Reclaim

The use of specific product names is illustrative only.  It is not intended to be a recommendation of one product over another, and is not intended to represent a complete listing of all products available. 

Document History

Status

Date

Action

Revised

05/03/2018

Medical Policy & Technology Assessment Committee (MPTAC) review. The document header wording updated from “Current Effective Date” to “Publish Date.” Removed MN criteria requiring failure of prior VNS treatment before RNS system. Updated Rationale and References sections.

Reviewed

08/03/2017

MPTAC review. Updated formatting in Position Statement section. Updated Rationale and References sections.

Reviewed

08/04/2016

MPTAC review. 

Reviewed

07/20/2016

Behavioral Health Subcommittee review. Updated Rationale and Reference sections.

 

01/01/2016

Updated Coding section with 01/01/2016 HCPCS changes; removed ICD-9 codes.

Reviewed

08/06/2015

MPTAC review. Updated Rationale and Reference sections.

 

01/01/2015

Updated Coding section with 01/01/2015 CPT changes; removed code 61875 deleted 12/31/2014.

Revised

08/14/2014

MPTAC review. Revised medically necessary criteria for cortical stimulation devices regarding the use of VNS. Updated Rationale section.

Revised

05/15/2014

MPTAC review. Revised document title to include cortical and cerebellar stimulation. Added medically necessary criteria for deep brain stimulation in individuals with incapacitating tremor from Parkinson’s disease. Added new medically necessary and investigational and not medically necessary position statements addressing cortical stimulation. Updated Rationale, Coding and Reference sections.

 

01/01/2014

Updated Coding section with 01/01/2014 HCPCS changes.

Reviewed

05/09/2013

MPTAC review. Rationale and Reference sections updated.

Reviewed

05/10/2012

MPTAC review. Rationale and References updated.

Reviewed

05/19/2011

MPTAC review. Rationale and References updated.

Revised

05/13/2010

MPTAC review. Clarified the position statement for medically necessary criteria. Added dystonia to investigational and not medically necessary statement regarding DBS for other causes. Rationale, Background, Definitions, Coding and References updated.

 

08/27/2009

Added Unified Parkinson's Disease Rating Scale (UPDRS) to the definitions; updated bibliography.

Reviewed

05/21/2009

MPTAC review. Rationale, coding and references updated.

Reviewed

05/15/2008

MPTAC review. References updated.

 

02/21/2008

The phrase "investigational/not medically necessary" was clarified to read "investigational and not medically necessary." This change was approved at the November 29, 2007 MPTAC meeting.

Reviewed

05/17/2007

 MPTAC review. References and Rationale updated. Coding updated; removed HCPCS E0752, E0754, E0756, E0757, and E0758 deleted 12/31/2005. 

Reviewed

06/08/2006

MPTAC review. References and coding updated. 

 

01/01/2006

Updated coding section with 01/01/2006 CPT/HCPCS changes

 

11/17/2005

Added reference for Centers for Medicare and Medicaid Services (CMS) – National Coverage Determination (NCD).

Revised

07/14/2005

MPTAC review. Revision based on Pre-merger Anthem and Pre-merger WellPoint Harmonization.

Pre-Merger Organizations

Last Review Date

Document Number

Title

 

Anthem, Inc.

 

06/16/2003

SURG.00026

Electrical Stimulation – Deep Brain, Cerebellar

WellPoint Health Networks, Inc.

04/28/2005

3.10.01

Deep Brain Stimulation for Tremor