|Home | About | Journals | Submit | Contact Us | Français|
A 55-year-old man who was in his usual state of good health jogged a short distance to his car as he was leaving a restaurant. As he sat down in the driver's seat, he experienced the sudden onset of a severe left occipital headache that was at maximal severity at onset and was associated with nausea and an episode of emesis. He experienced no photophobia, phonophobia, neck stiffness, or focal neurologic symptoms or signs. He had no history of headache. He presented to the emergency department at an outside institution about 20 minutes after onset. Findings on neurologic examination were normal, and he was not hypertensive.
Reversible cerebral vasoconstriction syndrome encompasses a number of different headache syndromes, including Call-Fleming syndrome, isolated benign cerebral vasculitis, benign acute cerebral angiopathy, CNS pseudovasculitis, benign angiopathy of the CNS, postpartum angiopathy, migrainous vasospasm, migraine angiitis, TCH with reversible vasospasm, and drug-induced cerebral vasculopathy or angiopathy.2 It is characterized by recurrent TCHs within a 7- to 14-day period and multifocal areas of cerebral vasoconstriction that often normalize within 12 weeks. Patients may have TCH with or without neurologic symptoms or signs.
Diagnostic criteria for RCVS include the following: (1) documentation by transfemoral angiography, CTA, or MRA of multifocal segmental cerebral artery vasoconstriction; (2) no evidence of aneurysmal SAH; (3) normal or near-normal findings on CSF analysis (protein level, <80 mg/dL; white blood cells, <10/μL; normal glucose level); (4) severe, acute headaches, with or without additional neurologic signs or symptoms; and (5) reversibility of angiographic abnormalities within 12 weeks after onset.
If death occurs before the follow-up studies are completed, autopsy rules out such conditions as vasculitis, intracranial atherosclerosis, and aneurysmal SAH, which can also manifest with headache and stroke.
In addition to diffuse, multifocal, segmental vasoconstriction on neurovascular imaging, other imaging abnormalities can be present. Changes consistent with PRES may be evident on MRI of the brain.11 Symmetric posterior watershed infarctions and multifocal intracerebral hemorrhages can also be seen.2 Ischemic stroke and TIAs usually occur within a week of the first TCH, whereas intracerebral hemorrhages most often occur in the second week. Cortical SAHs, such as that seen in this patient, have also been reported. In a study of 67 patients, 22% were found to have a cortical SAH, and 6% were found to have an intracerebral hemorrhage.10 Cortical SAHs were not implicated in causing diffuse vasoconstriction in any of these cases. The vascular constriction that occurs after an aneurysmal hemorrhage is typically delayed, occurring 1 to 2 weeks after the ictus, and most often involves the major cerebral arteries at the base of the brain.
Thunderclap headache can be the presenting symptom of numerous conditions. Often underdiagnosed, RCVS should be considered in patients presenting with recurrent TCH, PRES, a cortical SAH, or posterior watershed infarctions. Prompt noninvasive cerebrovascular imaging is required, and rapid diagnosis is of paramount importance because of the risk of ischemic stroke and intracerebral hemorrhage within the first 2 weeks after onset. Because up to 20% of cases may escape detection on initial noninvasive vascular imaging, catheter-based angiography may be considered in patients with recurrent TCH within a 7- to 14-day period when a high index of suspicion exists. Although the outcome of RCVS is benign in most patients, serious neurologic injury can occur. Recognizing triggers and initiating appropriate treatment can prevent serious complications.
We thank Dr Timothy Ingall for his help and guidance in preparing the submitted manuscript.
See end of article for correct answers to questions.
Correct answers: 1. e, 2. a, 3. b, 4. d, 5. e