Cardiac cephalalgia: one case with cortical hypoperfusion in headaches and literature review

BACKGROUND: Cardiac cephalalgia (CC) is a rare disease occurring during an episode of myocardial ischemia and relieved by nitroglycerine. Though more than 30 cases of CC have been reported since 1997, the mechanism is yet obscure. Herein, a case of CC is presented and discussed in relevance with previous literature to propose a novel hypothesis about the mechanism of CC. METHOD: A CC patient with cortical hypoperfusion during headache attacks was presented, which has never been reported. All published cases of CC via PubMed (http://www.ncbi.nlm.nih.gov/pubmed) in English literature, between 1997 and 2016, were reviewed. RESULTS: A patient suffering from CC presented a cerebral hypoperfusion during a headache attack. This phenomenon had not been observed since CC was introduced in 1997. The literature review summarized the clinical presentations, neuroimaging features, ECG, and coronary angiography features of 35 CC patients. CONCLUSION: Based on the phenomenon of hypoperfusion in the event of a headache, the vessel constriction hypothesis was proposed including two potential physiological mechanisms underlying the pathophysiology of CC.