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A proposal for revision of hemicrania continua diagnostic criteria based on critical analysis of 62 patients

Background: Misdiagnosis of hemicrania continua (HC) is common. It is diagnosed according to the criteria of the International Headache Society (IHS), but in clinical practice, these criteria seem too restrictive and many hemicrania continua-like headaches remain unclassified. Subjects and methods: We retrospectively studied the patients fulfilling the IHS criteria for HC. In addition, we identified a group of patients who met all criteria minus cranial autonomic features (HC-A) or minus a complete response to indomethacin (HC-I). We compared epidemiological profiles, clinical features and therapeutic responses to drug(s) between the groups. In addition, we reviewed the literature and critically analysed the diagnostic criteria for HC. Results: Sixty-two patients with a putative diagnosis of HC were identified. Thirty patients (48%) fulfilled the IHS criteria for HC. Thirty-two (52%) patients failed to satisfy one of the features of HC. Thirteen patients (21%) did not show any evidence of cranial autonomic feature during the episodes of the exacerbations (HC-A). Another 19 patients (31%) did not show a complete response to indomethacin (HC-I). Epidemiological and clinical features of all three groups were compared. None of the differences were statistically significant. In review of the literature, we noted that cranial autonomic features, a sense of restlessness/agitation during exacerbations, and response to indomethacin (marked to complete) are the specific features of HC. However, these features may not all be present in all cases. Conclusion: Present diagnostic criteria are too restrictive. We suggest adding the site of pain in the criteria, as described for trigeminal autonomic cephalalgias. Cranial autonomic features, a sense of restlessness during exacerbations, and marked to complete response to indomethacin are three characteristics features of HC. We suggest that the presence of any two may be sufficient to diagnose HC.