According to the National Institute of Neurological Disorders and Stroke, headache is the most commonly experienced form of pain.1 Of more than 150 types of headache listed in the International Classification of Headache Disorders (ICHD), tension-type headache (60%-80%) and migraine (15%) have the highest prevalence.2 Although other types of headache are much less common, an accurate diagnosis is essential to inform treatment strategy, particularly with headache that may be secondary to a potentially life-threatening medical condition.

In a March 2019 review published in the Medical Clinics of North America, investigators summarized evidence on nonmigraine headache and facial pain.3

Secondary headaches

Cervical artery dissection (CEAD) is a leading cause of ischemic stroke in young adults, for whom traditional risk factors are not the underlying etiology. Although CEAD is accompanied with symptoms similar to migraine or musculoskeletal pain, including headache and neck pain, patients typically have focal neurologic signs as well. “Dissection of the vertebral artery may result in cerebellar, brain stem (lateral medullary), or occipital lobe infarct, and dissection of the carotid artery may present with a partial Horner syndrome (ptosis and miosis) in addition to hemispheric strokes and dysautonomia,” noted the review authors.3

Migraine is one of the risk factors for CEAD, and people with migraine comprise one-third of cases of CEAD. Other risk factors include fibromuscular and connective tissue disorders and possibly recent infection or minor cervical trauma. “Pathognomonic radiodiagnostic findings include dissection flaps and ‘pearl-and-string’ sign.”3 Antiplatelet therapy is typically used to treat CEAD recurrence, and vascular surgery may be indicated in some cases.

Cerebral venous thrombosis is another cause of stroke that affects predominantly younger patients. The mean age of those with cerebral venoius thrombosis is 39 years, with women comprising an estimated 74% of these cases. Headache and seizure are present in 90% and 40% of cases of cerebral venous thrombosis, respectively.3 Other common clinical manifestations include paresis, papilledema, altered mental state, aphasia, diplopia, visual deficits, and stupor or coma.3,4 “Multiple venous sinuses are involved in almost half of cases, with the superficial veins of the superior sagittal sinus and transverse sinus most commonly involved,” noted the review authors.3

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Risk factors include thrombophilia, pregnancy/puerperium, oral contraceptive use, malignancy, infection, and dehydration. Treatment is with intravenous heparin in most cases, and thrombectomy/thrombolysis may be warranted in severe cases. With mortality rates of 6% to 20%, treatment in a stroke center is ideal, as such interventions have been associated with a 14% reduction in mortality.3

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by alternating constriction and dilation of the cerebral arteries, with “thunderclap” headaches, a term that refers to severe and explosive headaches that reach maximal intensity within 1 minute of onset as the primary symptom. These headaches recur over a period of 1 to 3 weeks and may be accompanied by nausea, vomiting, photophobia, blurred vision, and confusion.3,5 Sexual activity, Valsalva maneuvers, and straining have been reported as triggers for these headaches in some cases.

“Although the disease is self-limiting, there is a high risk [for] neurologic sequelae from intracranial hemorrhage, infarction, or edema. Initial arterial imaging peaks 2 to 3 weeks after symptom onset, and as such, normal imaging on presentation should be repeated.”

Risk factors for RCVS include nonaneurysmal subarachnoid hemorrhage, neurosurgical procedures, puerperium, vasoactive drugs, and certain conditions such as pheochromocytoma and porphyria.5 In addition to supportive measures and discontinuation of any offending agent, calcium channel blockers are often used to prevent vasospasm in patients with RCVS.