Man With Constant, Daily Headache Pain, Photophobia, Phonophobia, and Nausea
History: A 66-year-old man with long-standing history of chronic neuralgia of the right occipital nerve presents with constant, daily pain of variable intensity associated with photophobia, phonophobia, and frequent nausea. When the patient was 60 years old, he was in a motor vehicle collision (MVC). Acute medical workup performed immediately after the accident, which included a neurology consult, was negative for neurologic abnormality or injury to the cervical spine or cranium. Shortly after the MVC, the patient began to complain of persistent neck “stiffness” without neck or headache pain.
Twenty-eight months after the MVC, the patient developed severe right occipital pain that referred into the right parietal, frontal, and supraorbital regions, which persisted as constant pain for 90 days before spontaneously resolving without medical intervention. Three months later, 2 years and 10 months after the MVC, when the patient was 63 years old, his headache returned. As noted, the headache was associated with photophobia, phonophobia, and frequent nausea. The patient noted that the pain is constant and present all day, every day. It has never resolved without treatment.
At baseline, the patient has no pain-free days. The patient is taking Fioricet (a combination of butalbital 50 mg, acetaminophen 325 mg, and caffeine 40 mg), two tablets every 4 hours while awake, and Lortab (hydrocodone 10 mg/acetaminophen 650 mg) every 6 hours around the clock to manage his headache pain. This represents a total of 6,500 mg of acetaminophen per day, which exceeds the recommended maximum daily dose of 4,000 mg per day for a patient with normal liver function and previously raised serious concerns about the patient’s long-term health when these medications were his only treatment for his headache pain.
The patient’s medical history is positive for tension-type headache in adolescence, which is not similar to current complaints and resolved in young adulthood. The patient’s history is negative for migraine, vertigo, and blurred vision.
The patient’s family history is positive for Parkinson’s disease, bipolar disorder, depression, hypertension, and type 2 diabetes. His family history is negative for migraine. Physical examination is positive for tinel’s sign over the right occipital nerve, just inferior to the occipital protuberance and lateral to midline. Physical examination is negative for rhinorrhea, abnormal lacrimation, hyperhidrosis, facial flushing, and conjunctival erythema. Neurologic exam, including cranial nerves, is negative for deficits.
The patient has been enrolled in the author’s pain clinic, receiving repeated neural blockade of the greater occipital nerve (GON) and lesser occipital nerve (LON) for more than 3 years. After each procedure, the patient reports complete relief of his headache pain and associated symptoms for 5½ months, followed by a sudden return to pre-procedure baseline headache pain.
During the period of post-procedure analgesia, the patient reports that he is pain free and able to completely eliminate the need for all headache pain medication. In addition to pain relief, the patient also reports complete cessation of photophobia, phonophobia, and nausea during the post-procedure analgesia period.
Neuralgia of the GON or LON is a benign, extracranial cause of headache pain that has been described in the literature for more than 60 years. In the classic “neuralgia” presentation, GON typically presents as sudden, recurring hemicranial pain, often associated with tearing of the eye, flushing of the face, alteration of sweating pattern, and occlusion of the ipsilateral nasal passage.1,2 However, neuralgia of the GON and/or LON is an often-overlooked cause of chronic headache, especially when presenting in the context of occipital neuralgia with migraine headache.
The key to diagnosis is the presence of neuralgia-type pain, which is described as brief, sharp, lancinating pain in the distribution of the involved nerve.1 Blurred vision, rhinorrhea, and vertigo may be present, but are less common.2 In patients with chronic pain, there is usually a continuous, aching pain in the distribution of the occipital nerve, and sometimes the ophthalmic division of the trigeminal nerve.1,3,4 This pain may present episodically for intermittent, recurring periods of hours to days, or constantly for months or years at a time. The cardinal feature and diagnostic criteria are complete relief of pain following local anesthetic blockade of either the C2 nerve root or the greater and/or lesser occipital nerves.1,2,5,6
With minimal response from the patient’s medications, neuralgia of the GON was suspected. As noted, complete relief of symptoms following neural blockade of the GON and/or LON with local anesthesia and steroid will confirm the diagnosis. This technical report describes a novel technique for simultaneously blocking the GON and LON (see sidebar).
The GON and LON originate from the C2 nerve root.2 The relationship between occipital neuralgia and migraine is often poorly appreciated. However, there is a growing understanding that irritation or inflammation of the greater occipital nerve can produce referred pain in diverse cranial structures, not only within the C2 distribution but also within other nerves as well—particularly the ophthalmic division of the trigeminal nerve.4 Occipital neuralgia is also associated with migraine, either as a potential trigger or a late complication.3,5 In one study of 383 patients diagnosed with migraine headaches, 184 (48%) were found to have headaches caused by irritation of the GON that could be arrested by injecting the ipsilateral GON with local anesthetic.3
The mechanism by which irritation of the occipital nerves can be associated with migraine derives from the trigeminocervical complex. As reported by Bartsch et al, “neurons in the trigeminocervical complex are the major relay neurons for nociceptive, afferent input from the meninges and cervical structures; therefore, they are the neural substrates of head pain.”7 Stimulation of trigeminally innervated intracranial structures, such as supratentorial dura mater and large cranial vessels, evoke painful sensations, implying that afferent input from dural structures is the likely neural substrate in head pain and migraine.7 Nociceptive input from the dura mater is transmitted by small-diameter A and C fiber afferents in the ophthalmic division of the trigeminal nerve to nociceptive second-order neurons in the superficial and deep layers of the medullary dorsal horn of the trigeminocervical complex.7 Occipital and suboccipital structures, such as vessels and the dura mater of the posterior fossa, deep paraspinal neck muscles, upper cervical zygapophyseal (facet) joints, and ligaments, have nociceptive inflow that is also mediated by small-diameter afferent fibers, which synapse with nociceptive second-order neurons in the trigeminocervical complex.4,7 Thus, there is a direct coupling between meningeal afferents and cervical afferents in the spinal dorsal horn.8