Medical Policy: 07.01.59
Original Effective Date: November 2000
Reviewed: August 2016
Revised: August 2016
Benefit determinations are based on the applicable contract language in effect at the time the services were rendered. Exclusions, limitations or exceptions may apply. Benefits may vary based on contract, and individual member benefits must be verified. Wellmark determines medical necessity only if the benefit exists and no contract exclusions are applicable. This medical policy may not apply to FEP. Benefits are determined by the Federal Employee Program.
This Medical Policy document describes the status of medical technology at the time the document was developed. Since that time, new technology may have emerged or new medical literature may have been published. This Medical Policy will be reviewed regularly and be updated as scientific and medical literature becomes available.
Deep brain stimulation (DBS) is used as an alternative to permanent neuroablation procedures for control of essential tremor (ET) and Parkinson disease (PD). DBS is also being evaluated for the treatment of variety of other neurological and psychiatric disorders, including but not limited to epilepsy, dystonia, other movement disorder, cluster headache, Tourette syndrome, depression, and obsessive compulsive disorder (OCD).
DBS involves the stereotactic placement of an electrode into the brain (ie, ventral intermediate nucleus (Vim) of the thalamus, globus pallidus (GPi), or subthalamic nucleus (STN)). The electrode is initially attached to a temporary transcutaneous cable for short-term stimulation to validate treatment effectiveness. Several days later, the patient 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, use of bilateral stimulation using 2 electrode arrays has also been investigated in patients with bilateral, severe symptoms. After implantation, noninvasive programming of the neurostimulator can be adjusted to the patient's symptoms. This feature may be important for patients 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 adverse effects of neurostimulation, such as dysarthria, disequilibrium, or involuntary movements.
Deep brain stimulation (DBS) has been investigated as an alternative to thalamotomy and pallidotomy. The technique has been most thoroughly investigated as an alternative to thalamotomy for unilateral control of essential tremor (ET) and tremor associated with Parkinson disease (PD). More recently, there has been research interest in the use of DBS of the globus pallidus or subthalamic nucleus as a treatment of other parkinsonian symptoms, such as rigidity, bradykinesia, or akinesia. Another common morbidity associated with PD is the occurrence of motor fluctuations, referred to as "on and off" phenomena, related to the maximum effectiveness of drugs (ie, “on” state) and the nadir response during drug troughs (ie, “off”state). In addition, levodopa, the most commonly used anti-Parkinson drug, may be associated with disabling drug-induced dyskinesias. Therefore, the optimal pharmacologic treatment of PD may involve a balance between optimal effects on PD symptoms versus the appearance of drug-induced dyskinesias. The effect of DBS on both PD symptoms and drug-induced dyskinesias has also been studied.
Based on review of the medical literature there is sufficient evidence that deep brain stimulation (DBS) of the ventral intermediate nucleus (Vim) of the thalamus resulted in clinically significant tremor suppression and that outcomes after DBS were at least as good as thalamotomy. Subsequent studies reporting long-term follow up found that tremors were effectively controlled 5 to 6 years after DBS. The evidence is sufficient to determine that the use of DBS results in meaningful improvement in the net health outcome in the treatment of essential tremor.
Based on review of the medical literature the evidence from randomized controlled trials supports the conclusion that deep brain stimulation (DBS) of the globus pallidus (GPi) or subthalamic nucleus (STN) alleviates the motor fluctuations and dyskinesia associated with advanced Parkinson disease (PD). The evidence is sufficient to determine that the use of DBS results in a meaningful improvement in the net health outcome and is an effective therapeutic option for medically intractable symptoms of Parkinson disease.
Deep brain stimulation (DBS) has also been investigated in patients with primary dystonia, defined as a neurologic movement disorder characterized by involuntary muscle contractions, which force certain parts of the body into abnormal, contorted, and painful movements or postures. Dystonia can be classified according to age of onset, bodily distribution of symptoms, and cause. Age of onset can occur during childhood or during adulthood. Dystonia can affect certain portions of the body (focal dystonia and multifocal dystonia) or the entire body (generalized dystonia). Torticollis is an example of a focal dystonia. Primary dystonia is defined when dystonia is the only symptom unassociated with other pathology. Treatment options for dystonia include oral or injectable medications (ie, botulinum toxin) and destructive surgical or neurosurgical interventions (ie, thalamotomies or pallidotomies) when conservative therapies fail.
Based on review of the medical literature the evidence for deep brain stimulation (DBS) of the globus pallidus (GPi) or subthalamic nucleus (STN) in individuals who have primary dystonia includes randomized controlled trials and case series. Double-blind sham controlled studies generally found significantly better outcomes with active stimulation. The evidence is sufficient to determine that DBS results in a meaningful improvement in net health outcomes for children and adults with disabiling primary dystonia who do not respond to pharmacologic therapy or chemodenervation with botulinum toxin or other conservative therapies.
Deep brain stimulation (DBS) has been investigated in patients with chronic cluster headaches. Cluster headaches occur as episodic attacks of severe pain lasting from 30 minutes to several hours. The pain is usually unilateral and localized to the eye, temple, forehead, and side of the face. Autonomic symptoms that occur with cluster headaches include ipsilateral facial sweating, flushing, tearing, and rhinorrhea. Cluster headaches occur primarily in geno typical men and have been classified as vascular headaches that have been associated with high blood pressure, smoking, alcohol use, etc. However, the exact pathogenesis of cluster headaches is uncertain. Positron emission tomography scanning and magnetic resonance imaging (MRI) have shown the hypothalamic region may be important in the pathogenesis of cluster headaches. Alterations in hormonal/serotonergic function may also play a role. Treatment of cluster headaches includes pharmacologic interventions for acute episodes and prophylaxis and surgical procedures such gamma knife radiosurgery of the trigeminal nerve.
Based on review of the medical literature regarding DBS and treatment of cluster headaches, only a small number of patients have been studied. Additional randomized controlled studies are needed. The use of DBS for the treatment of cluster headaches is considered investigational as the current evidence on the efficacy is limited and inconsistent, and the evidence on safety shows that there are serious but well-known adverse effects.
Deep brain stimulation (DBS) has been investigated in patients with treatment resistant obsessive compulsive disorder (OCD). OCD is a disabling and potentially chronic mental disorder characterized by anxiety-provoking intrusive thoughts and repetitive behaviors. The literature on DBS for OCD consists of several RCTs and a number of uncontrolled studies. Most studies have small sample sizes. Meta-analysis of randomized trials may suggest that DBS may reduce symptoms for OCD, but larger trials are needed to confirm these preliminary findings and the impact of DBS on the net health benefit. The evidence is insufficient to determine the effects on health outcomes and therefore, DBS for the treatment of OCD is considered investigational.
Deep brain stimulation (DBS) has been investigated in patients with treatment resistant depression. The regions targeted for stimulation for this indication are also still being investigated. There is no compelling data that DBS is efficacious for the treatment of treatment resistant depression. Further randomized controlled clinical trials are needed to determine whether DBS is beneficial and at this time the evidence is insufficient to determine the effects on health outcomes. Deep brain stimulation is considered investigational for treatment resistant depression.
Tourette syndrome (TS) is a neurological disorder manifested by motor and phonic tics with onset during childhood. Deep brain stimulation has been investigated in patients who have disabling tics that are refractory to optimal medical management. The available evidence is preliminary and larger clinical trials are needed to determine whether DBS is beneficial for controlling tics in patients with TS. Also, the optimal target for DBS for the treatment of TS is unknown. The evidence is insufficient to determine the effects on health outcomes and therefore, DBS for the treatment of Tourette syndrome (TS) is considered investigational.
Tardive dyskinesia (TD) is a hyperkinetic movement disorder that appears with delayed onset, usually after prolonged receptor blocking agents, mainly the antipsychotic drugs (also called neuroleptics) and the antiemetic drug metoclopramide. TD has numerous clinical manifestations that include chorea, dystonia, akathisia, stereotyped behaviors and rarely tremor. Deep brain stimulation (DBS) has been investigated in patients who have permanent disabling TD that is unresponsive to pharmacologic treatment modalities. The evidence for DBS includes case reports and case series. Few studies were identified and they had small sample sizes (< 10 patients). Additional studies, especially RTCs or other controlled studies are needed. The evidence is insufficient to determine the effects on health outcomes and therefore, DBS for the treatment of tardive dyskinesia is considered investigational.
Multiple sclerosis (MS) is an autoimmune inflammatory demyelinating disease of the central nervous system (CNS) that is leading cause of disability in young adults. The course of MS is variable. For some, MS is a disease with one or two acute neurologic episodes and no further evidence of disease activity. In others, it is a chronic, relapsing, or progressive disease with an unpredictable clinical course that may span 10 to 20 years, during which time neurologic disability accumulates. Treatment directed at the progressive phase of MS is typically more difficult that treatment of relapsing forms for MS. Deep brain stimulation (DBS) has been investigated in patients who have multiple sclerosis (MS). Per review of the medical literature there was one randomized controlled trial identified. DBS had a positive impact on some outcomes but not others, and adverse events were reported. Additional RTCs are required to determine the impact of DBS on the net health outcome. The evidence is insufficient to determine the effects on health outcomes and therefore, DBS for the treatment of multiple sclerosis and is considered investigational.
Deep brain stimulation (DBS) has been investigated in patients who have refractory epilepsy. Patients with epilepsy whose seizures do not successfully respond to anti-seizure drug therapy are considered to have drug resistant epilepsy (DRE). This condition is also referred to as intractable, medically refractory or pharmacoresistant epilepsy. Per review of the medical literature there was one randomized controlled trial identified. DBS had a positive impact on some outcomes but not others, and adverse events were reported. Additional RTCs are required to determine the impact of DBS on the net health outcome. The evidence is insufficient to determine the effects on health outcomes and therefore, DBS for the treatment of epilepsy and is considered investigational.
Deep brain stimulation (DBS) has been investigated in patients who have anorexia nervosa/eating disorders, Alzheimer disease/dementia, Huntington disease, head or voice tremor, traumatic brain injury (TBI) and chronic pain. Per review of the medical literature the evidence includes case series and observational studies. Additional studies including randomized clinical trials or controlled studies are needed. The evidence is insufficient to determine the effects on health outcomes and therefore, DBS for the treatment of these indications is considered investigational.
DBS has been shown to be effective for the treatment of essential tremor (ET), advanced Parkinson disease (PD) and dystonia. Evidence for efficacy of DBS for Tourette syndrome, treatment of resistant depression, Obsessive compulsive disorder (OCD), Alzheimer disease/dementia, anorexia nervosa/eating disorders, chronic pain, epilepsy, tardive dyskinesia, multiple sclerosis, Huntington's disease, head or voice tremor and cluster headaches is based on experience with small number trials and small number of patients to also include case series and observational studies. In addition, the appropriate candidates and most effective target areas for DBS are under investigation. Additional randomized clinical trials or controlled studies with larger number of subjects are required to evaluate the role of DBS for these conditions.
Cerebellar stimulation/pacing is electrical stimulation using surgically implanted electrodes on the surface of the cerebellum and has been proposed as on 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 and therefore, is considered investigational.
2006 Guidelines from AAN on the treatment of Parkinson disease (PD) with motor fluctuations and dyskinesia states that deep brain stimulation (DBS) of the subthalamic nucleus (STN) may be considered to improve motor function and reduce off time, dyskinesia and medication usage (Level C). There is insufficient evidence to support or refute the efficacy of DBS of the GPi or VIM nucleus of the thalamus in reducing off time, dyskinesia or medication usage, or to improve motor function in PD patients.
2011 The American Academy of Neurology (AAN) published an updated guideline on the treatment of essential tremor (ET). There were no changes from the conclusions and recommendations of the 2005 practice parameters regarding DBS for ET. The guidelines stated bilateral DBS of the thalamic nucleus may be used to treat medically refractory limb tremor in both upper limbs (level C possibly effective), but there were insufficient data regarding the risk/benefit ration of bilateral versus unilateral DBS in the treatment of limb tremor. There was insufficient evidence to make recommendation regarding the use of thalamic for head or voice tremor (Level U, treatment is unproven). (This guideline was reaffirmed on April 30, 2014)
2013 Evidence based guideline from the AAN on the treatment of tardive syndromes states that the available evidence which consists of class IV studies comprising of case reports or small case series, is insufficient to support or refute pallidal deep brain stimulation for tardive syndromes. (This guideline was reaffirmed on July 16, 2016)
2007 American Psychiatric Association practice guideline for the treatment of patients with obsessive compulsive disorder states DBS may be recommended on the basis of individual circumstances.
In the 2013 guideline watch practice parameter for the treatment of patients with obsessive-compulsive disorder states DBS and ablative neurosurgical treatment for OCD should be performed only at sites with expertise in both OCD and these treatment approaches.
2010 American Psychiatric Association guideline on the treatment of major depressive disorder and the 2014 guideline watch, did not mention the use of deep brain stimulation (DBS) for the treatment of major depressive disorder.
The National Institute of Health and Care Excellence (NICE) has published Interventional Procedure Guidance documents on deep brain stimulation (DBS):
In 2006, NICE made the same statement for use of DBS for treatment of tremor and dystonia. Unilateral and bilateral stimulation of structures responsible for modifying movements, such as the thalamus, globus pallidus, and the subthalamic nucleus, which interact functionally with the substantia nigra, are included in both guidance statements.
In 2011 guidance states that there is evidence that DBS is efficacious in some patients who are refractory to other forms of pain control and that this procedure may be used provided normal arrangements are in place for clinical governance, consent and audit. Patients should be informed that DBS may not control their chronic pain symptoms and that possible risk associated with this procedure include small risk of death.
In 2011 guidance states that current evidence on the efficacy of DBS for intractable trigeminal autonomic cephalalgias (e.g. cluster headaches) is limited and inconsistent, and the evidence on safety shows that there are serious but well-known adverse effects. Therefore, this procedure should only be used with special arrangements for clinical governance, consent, and audit or research.
In 2012 guidance states that the evidence on the efficacy of the DBS for refractory epilepsy is limited in both quantity and quality. The evidence on safety shows that there are serious but well-known adverse effects. Therefore, the procedure should only be used with special arrangements for clinical governance, consent and audit or research.
In 2003 guidance states current evidence on the safety and efficacy of DBS for treatment of PD appears adequate to support the use of the procedure, provided that normal arrangements are in place for consent, audit, and clinical governance.
In 2006 guidance on the diagnosis and management of PD in primary and secondary care. With the evidence at that time, it was not possible to decide if the subthalamic nucleus or globus pallidus interna is the preferred target for DBS for people with PD, or whether one form of surgery is more effective or safer than the other. Based on level 3 or 4 evidence, NICE concluded that thalamic DBS may be considered an option in people with PD who predominantly have severe disabling tremor and where subthalamic nucleus stimulation cannot be performed.
The U.S. Food and Drug Administration (FDA) has approved the Activa® Tremor Control System, manufactured by Medtronic Corp, MN, for deep brain stimulation. While the original 1997 FDA-labeled indications were limited to unilateral implantation of the device for the treatment of tremor, in January 2002, the FDA-labeled indications were expanded to include bilateral implantation as a treatment to decrease the symptoms of advanced Parkinson’s disease that are not controlled by medication.
In April 2003, the labeled indications were expanded to include “unilateral or bilateral stimulation of the internal globus pallidus or subthalamic nucleus to aid in the management of chronic, intractable (drug refractory) primary dystonia, including generalized and/or segmental dystonia, hemidystonia, and cervical dystonia (torticollis) in patients seven years of age or above.” This latter indication received FDA approval through the Humanitarian Device Exemption process. The Activa Tremor Control System consists of the following components: the implantable pulse generator, the deep brain stimulator lead, an extension that connects the lead to the power source, a console programmer, a software cartridge to set electrical parameters for simulation, and a patient control magnet, which allows the patient to turn the pulse generator on and off, or change between high and low settings.
In February 2009, the FDA approved deep brain stimulation with the Reclaim® device (Medtronic, Inc.) via the Humanitarian Device Exemption (HDE) process for the treatment of severe obsessive-compulsive disorder (OCD).
The Vercise™ Deep Brain Stimulation system (Boston Scientific) is currently available in Europe, Israel,and Australia. Completion of a large U.S. multicenter trial (INTREPID) is expected in 2021.
In June 2015, the U.S. Food and Drug Administration approved the Brio Neurostimulation System (St. Jude Medical, Austin, TX) as a PMA (premarket approval) device for the following indications: 1) bilateral stimulation of the subthalmic nucleus (STN) as an adjunctive therapy to reduce some of the symptoms of advanced levodopa-responsive Parkinson’s disease that are not adequately controlled by medications; 2) unilateral or bilateral stimulation of the ventral intermediate (VIM) of the thalamus for the suppression of disabling upper extremity tremor in adult essential tremor patients whose tremor is not adequately controlled by medications and where the tremor constitutes a significant functional disability. The Brio is a rechargeable system.
Unilateral or bilateral deep brain stimulation (DBS) of the globus pallidus (GPi) or the subthalmic nucleus (STN) may be considered medically necessary in patients 7 years of age or older with chronic intractable (drug refractory) primary dystonia, including generalized and/or segmental dystonia, hemidystonia and cervical dystonia (torticollis)
Unilateral or bilateral deep brain stimulation (DBS) of the globus pallidus (GPi) or subthalmic nucleus (STN) may be considered medically necessary in individuals that have moderate to severe medically intractable idiopathic Parkinson’s disease with the following:
Unilateral deep brain stimulation (DBS) of the ventral intermediate nucleus (Vim) of the thalamus may be considered medically necessary in patients with disabling medically unresponsive tremor due to essential tremor (ET) or Parkinson disease (PD).
Bilateral deep brain stimulation of the ventral intermediate nucleus (Vim) of the thalamus may be considered medically necessary in patients with disabling, medically unresponsive tremor in both upper limbs due to essential tremor or Parkinson disease (PD).
Disabling, medically unresponsive tremor is defined as all of the following:
Replacement or revision of deep brain stimulator generator and/or lead/electrode(s) and/or programmer may be considered medically necessary for an individual that meets the above criteria and the existing generator/lead/electrode(s)/programmer is no longer under warranty and cannot be repaired.
Based on peer reviewed literature there is insufficient evidence to support the safety and effectiveness of deep brain stimulation (DBS), additional randomized clinical trials or controlled studies with larger number of subjects are required to evaluate the role of DBS for thees conditions and therefore is considered investigational, including but not limited to the following indications:
The use of cerebellar stimulation/pacing is considered investigational. Based on peer reviewed literature there is insufficient evidence to support the effectiveness of cerebellar stimulation/pacing on health outcomes and is therefore considered investigational.
UPDRS is a universal scale of Parkinson’s disease (PD) symptoms and it was created to comprehensively assess and document the exam of the patient with PD and be able to compare it with patient’s future follow up visits, or to communicate about the progression of the PD symptoms in each patient with other neurologists.
The UPDRS is made up of the 1) Mentation, Behavior, and Mood, 2) ADL and 3) Motor sections. These are evaluated by interview. Some sections require multiple grades assigned to each extremity. A total of 199 points are possible. 199 represents the worst (total) disability), 0--no disability.
Uncontrolled shaking or trembling, usually of one or both hands or arms, that worsens when basic movements are attempted. It is caused by abnormalities in areas of the brain that control movement and is not tied to an underlying disease (e.g. Parkinson’s disease).
Highly variable neurological movement disorder characterized by involuntary muscle contractions. Dystonia results from abnormal functioning of the basal ganglia, a deep part of the brain which helps control coordination of movement. These regions of the brain control the speed and fluidity of movement and prevent unwanted movements. Patients with dystonia may experience uncontrollable twisting, repetitive movements, or abnormal postures and positions. These can affect any part of the body, including the arms, legs, trunk, face and vocal cords. Dystonia can affect young children to older adults of all races and ethnicities.
Dystonia is the only sign, and secondary causes have been ruled out. Most primary dystonias are variable, have adult onset, and are focal or segmental in nature. However, there are specific primary dystonias with childhood or adolescent onset that have been linked to genetic mutations.
Most common form of Parkinson’s disease, and the cause essentially remains unknown. Parkinson’s disease is a progressive disorder that is caused by a degeneration of nerve cells in the part of the brain called the substantia nigra, which controls movement. These nerve cells die or become impaired, losing the ability to produce an important chemical called dopamine.
This is a disorder with symptoms similar to Parkinson’s, but is caused by medication side effects, different neurodegenerative disorders, Illness, or brain damage.
August 2016 - Annual Review, Policy Revised
September 2015 - Annual Review, Policy Revised
February 2015 - Policy Revised
October 2014 - Annual Review, Policy Revised
January 2014 - Annual Review, Revised and New Policy Created
January 2013 - Annual Review, Policy Renewed
January 2012 - Annual Review, Policy Renewed
February 2011 - Interim Review, Policy Revised
October 2010 - Annual Review, Policy Renewed
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