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Diagnosis and treatment can be complicated when the headache history includes evidence of central and peripheral causes of pain.
ASHEVILLE, NC—Although neurologists tend to classify disorders as problems of either the CNS or the peripheral nervous system, patients with headache may have symptoms that indicate the involvement of both systems, according to an overview provided at the Eighth Annual Scientific Meeting of the Southern Headache Society. Research has revealed anatomic connections between extracranial and intracranial spaces that could contribute to the generation of headaches. Thus, the central and peripheral nervous systems “do not have to be two separate spaces and two separate pathologies,” said Pamela Blake, MD, Director of the Headache Center of Greater Heights in Houston.
Problems With Prevention and Acute Treatment
One patient presented to Dr. Blake with an eight-year history of throbbing headaches and constant pain and tightness in the neck and occiput. The pain radiated to her temples and forehead two to four times per week with accompanying photophobia, phonophobia, and nausea. The patient also had mild allodynia. The frontal pain and accompanying symptoms were consistent with episodic migraine, but the allodynia and pain in the neck and occiput were not, said Dr. Blake. A possible diagnosis was episodic migraine without aura with chronic tension-type headaches and neck pain, she added. 
A 2017 study published in the Journal of Headache and Pain suggested that this headache type is problematic. Among 148 migraineurs, the researchers identified 100 patients who also had tension-type headache and chronic neck pain. Compared with healthy controls, these patients had less physical activity, less psychologic well-being, more perceived stress, and poorer self-rated health. Pain reduced these patients’ ability to perform physical activity, which could make treatment more difficult, according to the authors.
Patients with these symptoms have trigeminal and occipital pain. “These symptoms do not appear to be solely, or even primarily, central,” said Dr. Blake. The frontal pain responds to triptans, but the occipital pain, which is the more constant pain, does not. “Preventive medications do not work well in this population, and that’s why they have chronic headaches,” said Dr. Blake.
Physiologic and Pathophysiologic Mechanisms
Research by Schueler and colleagues suggested a potential physiologic explanation for combined central and peripheral involvement in headache. They applied a fluorescent tracer to proximally cut meningeal nerves in rat skulls and to distal branches of the spinosus nerve in human calvaria that was lined with dura mater. They observed that branches of the spinosus nerve travel “along the middle meningeal artery, supplying the dura, entering the cranial bone, and running through the calvarium,” said Dr. Blake. Branches of the spinosus nerve also “entered the tenderness junctions of the pericranial muscles, including in the neck.”
This work indicates a connection between intracranial and extracranial areas but does not shed light on the pathophysiology of a headache with central and peripheral symptoms, said Dr. Blake. In 2016, she and her colleagues took perivascular biopsies from healthy controls and subjects with chronic migraine and predominantly occipital headache. They found a significant increase in the expression of proinflammatory genes and a decrease in the expression of anti-inflammatory genes among migraineurs, compared with controls. “This was the first evidence of localized extracranial pathophysiology in chronic migraine,” said Dr. Blake.
This inflammation could result from compression of the occipital nerves. A 2013 study by Schmid et al found that progressive nerve compression results in chronic local and remote immune-mediated inflammation. Stress also can cause inflammation. “Many patients who present with occipital nerve compression headaches had the onset of their pain during a time of intense stress,” said Dr. Blake.
The Role of the Occipital Nerve
Occipital nerve compression headache is characterized by daily or near-daily pain in the distribution of the occipital nerve. Patients describe the pain as a tight, imploding pressure that sometimes radiates to frontal areas and becomes a throbbing pain. This headache rarely has a neuropathic component.
Allodynia is “almost a requirement of this diagnosis,” said Dr. Blake. The allodynia symptom checklist, however, does not capture it well in these patients because it focuses on pain in the trigeminal nerve distribution. Patients report that the back of the head is tender to the touch and that it hurts to rest the head on a pillow.
The cervical muscles compress the nerve and contribute to the symptoms as well. Patients often report that moving the head or neck exacerbates the pain. The headache also may have migrainous features. It takes skill and expertise to elicit an adequate history from these patients, said Dr. Blake. “Careful questioning is helpful. I often find … that a second visit is more helpful to obtain this history after reviewing the anatomy with the patient, reviewing this pathophysiology, and sending them back out to keep a careful log for two weeks.”
Nerve Blocks and Nerve Decompression
Occipital nerve blocks provide relief for these patients, but they may not be easy to administer. A large dorsal occipital nerve may be mistaken for the greater occipital nerve, for example. Physiologic abnormalities in some patients also can complicate this treatment.
Another effective treatment is nerve decompression surgery. Dr. Blake and colleagues conducted a retrospective review of patients who had undergone decompression of the greater occipital nerves at the point where they traverse the musculature of the posterior neck. The intervention provided complete relief for three to six years in one patient with new daily persistent headache and two patients with chronic posttraumatic headache. Two patients with chronic headache or migraine had partial relief. Surgery provided no relief for two patients with episodic migraine, one patient with chronic migraine, and one patient with chronic tension-type headache.
“In our experience, … 75% to 80% of patients experience a greater than 50% reduction in their headaches, measured by headache frequency and intensity,” said Dr. Blake. She and her colleagues compared outcomes between 18 chronic migraineurs with predominantly occipital pain who underwent surgical decompression of the occipital nerve and 23 patients who were referred for surgery but unable to receive it. In the surgical group, the number of predominantly occipital chronic migraine days per month decreased from 28.9 at baseline to 7.28 at a mean of 46 months later. The outcome in the control group did not change.
Correct patient selection “is the first, most important step” toward treatment success, said Dr. Blake. This process includes a preoperative psychologic evaluation that screens surgical candidates for somatic symptom disorder, mood disorders, a history of trauma, and catastrophizing. If indicated, neurologists may begin providing cognitive behavioral therapy or supportive psychotherapy before surgery. “We have a comprehensive program with postoperative management, including physical therapy and gradual taper of medications,” said Dr. Blake. Central migraine processes may contribute to headache in some of these patients. “Menstrual migraines are not going to go away with nerve decompression. Chronic migraine sometimes does not go away. This is a complex group of patients who definitely require a lot of follow-up.”
—Erik Greb
Suggested Reading
Blake P, Nir RR, Perry CJ, Burstein R. Tracking patients with chronic occipital headache after occipital nerve decompression surgery: a case series. Cephalalgia. 2018 Sep 14 [Epub ahead of print].
Krøll LS, Hammarlund CS, Westergaard ML, et al. Level of physical activity, well-being, stress and self-rated health in persons with migraine and co-existing tension-type headache and neck pain. J Headache Pain. 2017;18(1):46. Perry CJ, Blake P, Buettner C, et al. Upregulation of inflammatory gene transcripts in periosteum of chronic migraineurs: implications for extracranial origin of headache. Ann Neurol. 2016;79(6):1000-1013.
Schmid AB, Coppieters MW, Ruitenberg MJ, McLachlan EM. Local and remote immune-mediated inflammation after mild peripheral nerve compression in rats. J Neuropathol Exp Neurol. 2013;72(7):662-680.
Schueler M, Neuhuber WL, De Col R, Messlinger K. Innervation of rat and human dura mater and pericranial tissues in the parieto-temporal region by meningeal afferents. Headache. 2014;54(6):996-1009.
Diagnosis and treatment can be complicated when the headache history includes evidence of central and peripheral causes of pain.
Diagnosis and treatment can be complicated when the headache history includes evidence of central and peripheral causes of pain.
ASHEVILLE, NC—Although neurologists tend to classify disorders as problems of either the CNS or the peripheral nervous system, patients with headache may have symptoms that indicate the involvement of both systems, according to an overview provided at the Eighth Annual Scientific Meeting of the Southern Headache Society. Research has revealed anatomic connections between extracranial and intracranial spaces that could contribute to the generation of headaches. Thus, the central and peripheral nervous systems “do not have to be two separate spaces and two separate pathologies,” said Pamela Blake, MD, Director of the Headache Center of Greater Heights in Houston.
Problems With Prevention and Acute Treatment
One patient presented to Dr. Blake with an eight-year history of throbbing headaches and constant pain and tightness in the neck and occiput. The pain radiated to her temples and forehead two to four times per week with accompanying photophobia, phonophobia, and nausea. The patient also had mild allodynia. The frontal pain and accompanying symptoms were consistent with episodic migraine, but the allodynia and pain in the neck and occiput were not, said Dr. Blake. A possible diagnosis was episodic migraine without aura with chronic tension-type headaches and neck pain, she added.
A 2017 study published in the Journal of Headache and Pain suggested that this headache type is problematic. Among 148 migraineurs, the researchers identified 100 patients who also had tension-type headache and chronic neck pain. Compared with healthy controls, these patients had less physical activity, less psychologic well-being, more perceived stress, and poorer self-rated health. Pain reduced these patients’ ability to perform physical activity, which could make treatment more difficult, according to the authors.
Patients with these symptoms have trigeminal and occipital pain. “These symptoms do not appear to be solely, or even primarily, central,” said Dr. Blake. The frontal pain responds to triptans, but the occipital pain, which is the more constant pain, does not. “Preventive medications do not work well in this population, and that’s why they have chronic headaches,” said Dr. Blake.
Physiologic and Pathophysiologic Mechanisms
Research by Schueler and colleagues suggested a potential physiologic explanation for combined central and peripheral involvement in headache. They applied a fluorescent tracer to proximally cut meningeal nerves in rat skulls and to distal branches of the spinosus nerve in human calvaria that was lined with dura mater. They observed that branches of the spinosus nerve travel “along the middle meningeal artery, supplying the dura, entering the cranial bone, and running through the calvarium,” said Dr. Blake. Branches of the spinosus nerve also “entered the tenderness junctions of the pericranial muscles, including in the neck.”
This work indicates a connection between intracranial and extracranial areas but does not shed light on the pathophysiology of a headache with central and peripheral symptoms, said Dr. Blake. In 2016, she and her colleagues took perivascular biopsies from healthy controls and subjects with chronic migraine and predominantly occipital headache. They found a significant increase in the expression of proinflammatory genes and a decrease in the expression of anti-inflammatory genes among migraineurs, compared with controls. “This was the first evidence of localized extracranial pathophysiology in chronic migraine,” said Dr. Blake.
This inflammation could result from compression of the occipital nerves. A 2013 study by Schmid et al found that progressive nerve compression results in chronic local and remote immune-mediated inflammation. Stress also can cause inflammation. “Many patients who present with occipital nerve compression headaches had the onset of their pain during a time of intense stress,” said Dr. Blake.
The Role of the Occipital Nerve
Occipital nerve compression headache is characterized by daily or near-daily pain in the distribution of the occipital nerve. Patients describe the pain as a tight, imploding pressure that sometimes radiates to frontal areas and becomes a throbbing pain. This headache rarely has a neuropathic component.
Allodynia is “almost a requirement of this diagnosis,” said Dr. Blake. The allodynia symptom checklist, however, does not capture it well in these patients because it focuses on pain in the trigeminal nerve distribution. Patients report that the back of the head is tender to the touch and that it hurts to rest the head on a pillow.
The cervical muscles compress the nerve and contribute to the symptoms as well. Patients often report that moving the head or neck exacerbates the pain. The headache also may have migrainous features. It takes skill and expertise to elicit an adequate history from these patients, said Dr. Blake. “Careful questioning is helpful. I often find … that a second visit is more helpful to obtain this history after reviewing the anatomy with the patient, reviewing this pathophysiology, and sending them back out to keep a careful log for two weeks.”
Nerve Blocks and Nerve Decompression
Occipital nerve blocks provide relief for these patients, but they may not be easy to administer. A large dorsal occipital nerve may be mistaken for the greater occipital nerve, for example. Physiologic abnormalities in some patients also can complicate this treatment.
Another effective treatment is nerve decompression surgery. Dr. Blake and colleagues conducted a retrospective review of patients who had undergone decompression of the greater occipital nerves at the point where they traverse the musculature of the posterior neck. The intervention provided complete relief for three to six years in one patient with new daily persistent headache and two patients with chronic posttraumatic headache. Two patients with chronic headache or migraine had partial relief. Surgery provided no relief for two patients with episodic migraine, one patient with chronic migraine, and one patient with chronic tension-type headache.
“In our experience, … 75% to 80% of patients experience a greater than 50% reduction in their headaches, measured by headache frequency and intensity,” said Dr. Blake. She and her colleagues compared outcomes between 18 chronic migraineurs with predominantly occipital pain who underwent surgical decompression of the occipital nerve and 23 patients who were referred for surgery but unable to receive it. In the surgical group, the number of predominantly occipital chronic migraine days per month decreased from 28.9 at baseline to 7.28 at a mean of 46 months later. The outcome in the control group did not change.
Correct patient selection “is the first, most important step” toward treatment success, said Dr. Blake. This process includes a preoperative psychologic evaluation that screens surgical candidates for somatic symptom disorder, mood disorders, a history of trauma, and catastrophizing. If indicated, neurologists may begin providing cognitive behavioral therapy or supportive psychotherapy before surgery. “We have a comprehensive program with postoperative management, including physical therapy and gradual taper of medications,” said Dr. Blake. Central migraine processes may contribute to headache in some of these patients. “Menstrual migraines are not going to go away with nerve decompression. Chronic migraine sometimes does not go away. This is a complex group of patients who definitely require a lot of follow-up.”
—Erik Greb
Suggested Reading
Blake P, Nir RR, Perry CJ, Burstein R. Tracking patients with chronic occipital headache after occipital nerve decompression surgery: a case series. Cephalalgia. 2018 Sep 14 [Epub ahead of print].
Krøll LS, Hammarlund CS, Westergaard ML, et al. Level of physical activity, well-being, stress and self-rated health in persons with migraine and co-existing tension-type headache and neck pain. J Headache Pain. 2017;18(1):46. Perry CJ, Blake P, Buettner C, et al. Upregulation of inflammatory gene transcripts in periosteum of chronic migraineurs: implications for extracranial origin of headache. Ann Neurol. 2016;79(6):1000-1013.
Schmid AB, Coppieters MW, Ruitenberg MJ, McLachlan EM. Local and remote immune-mediated inflammation after mild peripheral nerve compression in rats. J Neuropathol Exp Neurol. 2013;72(7):662-680.
Schueler M, Neuhuber WL, De Col R, Messlinger K. Innervation of rat and human dura mater and pericranial tissues in the parieto-temporal region by meningeal afferents. Headache. 2014;54(6):996-1009.
ASHEVILLE, NC—Although neurologists tend to classify disorders as problems of either the CNS or the peripheral nervous system, patients with headache may have symptoms that indicate the involvement of both systems, according to an overview provided at the Eighth Annual Scientific Meeting of the Southern Headache Society. Research has revealed anatomic connections between extracranial and intracranial spaces that could contribute to the generation of headaches. Thus, the central and peripheral nervous systems “do not have to be two separate spaces and two separate pathologies,” said Pamela Blake, MD, Director of the Headache Center of Greater Heights in Houston.
Problems With Prevention and Acute Treatment
One patient presented to Dr. Blake with an eight-year history of throbbing headaches and constant pain and tightness in the neck and occiput. The pain radiated to her temples and forehead two to four times per week with accompanying photophobia, phonophobia, and nausea. The patient also had mild allodynia. The frontal pain and accompanying symptoms were consistent with episodic migraine, but the allodynia and pain in the neck and occiput were not, said Dr. Blake. A possible diagnosis was episodic migraine without aura with chronic tension-type headaches and neck pain, she added.
A 2017 study published in the Journal of Headache and Pain suggested that this headache type is problematic. Among 148 migraineurs, the researchers identified 100 patients who also had tension-type headache and chronic neck pain. Compared with healthy controls, these patients had less physical activity, less psychologic well-being, more perceived stress, and poorer self-rated health. Pain reduced these patients’ ability to perform physical activity, which could make treatment more difficult, according to the authors.
Patients with these symptoms have trigeminal and occipital pain. “These symptoms do not appear to be solely, or even primarily, central,” said Dr. Blake. The frontal pain responds to triptans, but the occipital pain, which is the more constant pain, does not. “Preventive medications do not work well in this population, and that’s why they have chronic headaches,” said Dr. Blake.
Physiologic and Pathophysiologic Mechanisms
Research by Schueler and colleagues suggested a potential physiologic explanation for combined central and peripheral involvement in headache. They applied a fluorescent tracer to proximally cut meningeal nerves in rat skulls and to distal branches of the spinosus nerve in human calvaria that was lined with dura mater. They observed that branches of the spinosus nerve travel “along the middle meningeal artery, supplying the dura, entering the cranial bone, and running through the calvarium,” said Dr. Blake. Branches of the spinosus nerve also “entered the tenderness junctions of the pericranial muscles, including in the neck.”
This work indicates a connection between intracranial and extracranial areas but does not shed light on the pathophysiology of a headache with central and peripheral symptoms, said Dr. Blake. In 2016, she and her colleagues took perivascular biopsies from healthy controls and subjects with chronic migraine and predominantly occipital headache. They found a significant increase in the expression of proinflammatory genes and a decrease in the expression of anti-inflammatory genes among migraineurs, compared with controls. “This was the first evidence of localized extracranial pathophysiology in chronic migraine,” said Dr. Blake.
This inflammation could result from compression of the occipital nerves. A 2013 study by Schmid et al found that progressive nerve compression results in chronic local and remote immune-mediated inflammation. Stress also can cause inflammation. “Many patients who present with occipital nerve compression headaches had the onset of their pain during a time of intense stress,” said Dr. Blake.
The Role of the Occipital Nerve
Occipital nerve compression headache is characterized by daily or near-daily pain in the distribution of the occipital nerve. Patients describe the pain as a tight, imploding pressure that sometimes radiates to frontal areas and becomes a throbbing pain. This headache rarely has a neuropathic component.
Allodynia is “almost a requirement of this diagnosis,” said Dr. Blake. The allodynia symptom checklist, however, does not capture it well in these patients because it focuses on pain in the trigeminal nerve distribution. Patients report that the back of the head is tender to the touch and that it hurts to rest the head on a pillow.
The cervical muscles compress the nerve and contribute to the symptoms as well. Patients often report that moving the head or neck exacerbates the pain. The headache also may have migrainous features. It takes skill and expertise to elicit an adequate history from these patients, said Dr. Blake. “Careful questioning is helpful. I often find … that a second visit is more helpful to obtain this history after reviewing the anatomy with the patient, reviewing this pathophysiology, and sending them back out to keep a careful log for two weeks.”
Nerve Blocks and Nerve Decompression
Occipital nerve blocks provide relief for these patients, but they may not be easy to administer. A large dorsal occipital nerve may be mistaken for the greater occipital nerve, for example. Physiologic abnormalities in some patients also can complicate this treatment.
Another effective treatment is nerve decompression surgery. Dr. Blake and colleagues conducted a retrospective review of patients who had undergone decompression of the greater occipital nerves at the point where they traverse the musculature of the posterior neck. The intervention provided complete relief for three to six years in one patient with new daily persistent headache and two patients with chronic posttraumatic headache. Two patients with chronic headache or migraine had partial relief. Surgery provided no relief for two patients with episodic migraine, one patient with chronic migraine, and one patient with chronic tension-type headache.
“In our experience, … 75% to 80% of patients experience a greater than 50% reduction in their headaches, measured by headache frequency and intensity,” said Dr. Blake. She and her colleagues compared outcomes between 18 chronic migraineurs with predominantly occipital pain who underwent surgical decompression of the occipital nerve and 23 patients who were referred for surgery but unable to receive it. In the surgical group, the number of predominantly occipital chronic migraine days per month decreased from 28.9 at baseline to 7.28 at a mean of 46 months later. The outcome in the control group did not change.
Correct patient selection “is the first, most important step” toward treatment success, said Dr. Blake. This process includes a preoperative psychologic evaluation that screens surgical candidates for somatic symptom disorder, mood disorders, a history of trauma, and catastrophizing. If indicated, neurologists may begin providing cognitive behavioral therapy or supportive psychotherapy before surgery. “We have a comprehensive program with postoperative management, including physical therapy and gradual taper of medications,” said Dr. Blake. Central migraine processes may contribute to headache in some of these patients. “Menstrual migraines are not going to go away with nerve decompression. Chronic migraine sometimes does not go away. This is a complex group of patients who definitely require a lot of follow-up.”
—Erik Greb
Suggested Reading
Blake P, Nir RR, Perry CJ, Burstein R. Tracking patients with chronic occipital headache after occipital nerve decompression surgery: a case series. Cephalalgia. 2018 Sep 14 [Epub ahead of print].
Krøll LS, Hammarlund CS, Westergaard ML, et al. Level of physical activity, well-being, stress and self-rated health in persons with migraine and co-existing tension-type headache and neck pain. J Headache Pain. 2017;18(1):46. Perry CJ, Blake P, Buettner C, et al. Upregulation of inflammatory gene transcripts in periosteum of chronic migraineurs: implications for extracranial origin of headache. Ann Neurol. 2016;79(6):1000-1013.
Schmid AB, Coppieters MW, Ruitenberg MJ, McLachlan EM. Local and remote immune-mediated inflammation after mild peripheral nerve compression in rats. J Neuropathol Exp Neurol. 2013;72(7):662-680.
Schueler M, Neuhuber WL, De Col R, Messlinger K. Innervation of rat and human dura mater and pericranial tissues in the parieto-temporal region by meningeal afferents. Headache. 2014;54(6):996-1009.