Teaching case: Occipital Neuralgia in a Young Patient

Case Presentation A 27-year-old left-handed male, with medical history significant only for exploratory laparotomy for intussusceptions in childhood, was initially seen by the neurology service at our institution for a 10-year history of headaches. He was seen in consultation specifically for consideration of occipital nerve stimulator. He did have a history of 2 MVAs proximate to the time of headache onset. In the first, he was a restrained driver of a vehicle that rolled over, causing loss of consciousness for a few seconds without posttraumatic amnesia or other injuries. In the second, which was about a year after the first, there was no loss of consciousness, but he did suffer a neck strain with residual neck pain, which went away after several weeks. It was at this time he started to experience new daily persistent headache. The headache quality was described as variable. The less intense headache, which was more common, was described as mild posterior pressure sensation located over the bioccipital-nuchal region. It had an average intensity of 1 to 2/10 on a verbal numeric score (VNS). The headache would exacerbate frequently to a more intense headache, which was almost always left hemicranial, rarely right, which was severe and sharp, without nausea, vomiting, aura, or prodrome, but with mild phono but no photophobia. This was rated as a 10/10 on VNS. Triggers included caffeine, sweets, onions, hot dogs, and laughing, which would trigger a headache within a few seconds, but not a true Valsalva. His symptoms were worse with heat or warm temperatures and watching television. He had a vision check and was prescribed new glasses for myopia, which helped his vision but had no effect on his headache. He had a cousin with similar symptoms, but no first degree relative. He did have a history of motion sickness, fainting, but did not have cold hands or feet. His sleep was good, mood was “okay,” and energy level was appropriate. He worked as an installer of industrial air ducts and was a volunteer fireman. Prior to being seen by Neurology at our institution, the patient was being managed by a neurologist in the community. His medications were eletriptan, indomethacin, nadolol, and topiramate. He was trialed on divalproex sodium, which exacerbated his headache, and was discontinued. On initial examination, his vitals revealed blood pressure on the low range of normal, completely normal neurological, vascular, and musculoskeletal exams with the exception of tenderness bilaterally over the occipital nerves. Initial management included referral for an MRI examination to rule out Chiari malformation, and gradual reduction of nadolol. On followup examination, his BP was 86/51 with a pulse of 40, and his nadolol was tapered off. He continued to have tenderness bilaterally over the occipital nerves and underwent occipital nerve block using 1% lidocaine and 20 mg Depo-Medrol . He was started on Indocin SR for exacerbations and given famotidine for prophylaxis. He was tapered off topiramate. Patient was called the next day after block to report that he had no headache, but returned 6 days later with a lesser intensity. He then continued to have a low background daily headache with more intense exacerbations several times per week. He was then diagnosed with bilateral hemicrania continua and started on zonisamide. His baseline CBC and liver enzymes were normal. He continued to respond to indomethacin at 50 BID and his zonisamide was raised to 200 mg at bedtime. Based on the persistence of his symptoms, he was then referred to the Pain Clinic for evaluation and consideration for peripheral nerve stimulation. Evaluation in the pain clinic revealed persistent tenderness in the occipital region bilaterally, left greater than right as well as tenderness over the spinous processes from C2 to C6-7 with a normal neurological and vascular examination. The patient and his wife were then presented with information regarding occipital nerve stimulation, including the risks and benefits of the procedure. They were explained that trial would be necessary to assess effectiveness followed by permanent placement. They elected to proceed with trial, feeling that something different needed to be attempted given persistence of symptoms and its effect on his function. Psychological clearance, as required by most insurance companies, was then obtained as was prior authorization from his insurance company. The patient then underwent surgical placement of a percutaneously placed Medtronic “Pisces Quad” lead over the greater occipital nerve, bilaterally (see the Figure) without complication. The stimulation paresthesias were tolerable and provided excellent coverage of his headache bilaterally intra-operatively. The trial leads were then secured to the skin and connected to an external generator placed in a belt pack. The patient was admitted overnight for pain control, monitoring, and for education regarding the use of the system. Prior to discharge the next morning, his stimulator system was reprogrammed to optimize stimulator coverage of his occipital nerves bilaterally. He was instructed how to use the Medtronic “MyStim” remote control system and given limited control of certain parameters to customize stimulation during his trial period outside the hospital. He was