Abstract:-Hemicrania continua (HC) is an uncommon primary headache first described as a syndrome in 1984. Being quite unusual, its clinical characterization still demands better description. The aim of this study is to present the main clinical characteristics of 10 patients with the diagnosis of HC seen in a tertiary center, critically discussing their main features. All subjects had strictly unilateral headache without side shift and absolute response to indomethacin. Seven patients (70%) presented autonomic features… Show more
“…Jabs and jolts like pain (stabbing headache) are important associated pains during exacerbation phase. They were noted in 20%–41% patients with HC in different case series 6,14,37. However, this prevalence matches with the normal prevalence of stabbing headache in the general population.…”
Section: Other Associated Headache Disorderssupporting
Hemicrania continua (HC) is an indomethacin-responsive primary headache disorder which is currently classified under the heading of trigeminal autonomic cephalalgias (TACs). It is a highly misdiagnosed and underreported primary headache. The pooled mean delay of diagnosis of HC is 8.0 ± 7.2 years. It is not rare. We noted more than 1000 cases in the literature. It represents 1.7% of total headache patients attending headache or neurology clinic. Just like other TACs, it is characterized by strictly unilateral pain in the trigeminal distribution, cranial autonomic features in the same area and agitation during exacerbations/attacks. It is different from other TACs in one aspect. While all other TACs are episodic, HC patients have continuous headaches with superimposed severe exacerbations. The central feature of HC is continuous background headache. However, the patients may be worried only for superimposed exacerbations. Focusing only on exacerbations and ignoring continuous background headache are the most important factors for the misdiagnosis of HC. A large number of patients may have migrainous features during exacerbation phase. Up to 70% patients may fulfill the diagnostic criteria for migraine during exacerbations. Besides migraine, its exacerbations can mimic a large number of other primary and secondary headaches. The other specific feature of HC is a remarkable response to indomethacin. However, a large number of patients develop side effects because of the long-term use of indomethacin. A few other medications may also be effective in a subset of patients with HC. Various surgical interventions have been suggested for patients who are intolerant to indomethacin. Several aspects of HC are still not defined. There is a great heterogeneity in types of patients or articles on the HC in the literature. Diagnostic criteria have been modified several times over the years. The current diagnostic criteria are too restrictive in some aspects. We suggest a more accommodating type of criteria for the appendix of International Classification of Headache Disorder (ICHD).
“…Jabs and jolts like pain (stabbing headache) are important associated pains during exacerbation phase. They were noted in 20%–41% patients with HC in different case series 6,14,37. However, this prevalence matches with the normal prevalence of stabbing headache in the general population.…”
Section: Other Associated Headache Disorderssupporting
Hemicrania continua (HC) is an indomethacin-responsive primary headache disorder which is currently classified under the heading of trigeminal autonomic cephalalgias (TACs). It is a highly misdiagnosed and underreported primary headache. The pooled mean delay of diagnosis of HC is 8.0 ± 7.2 years. It is not rare. We noted more than 1000 cases in the literature. It represents 1.7% of total headache patients attending headache or neurology clinic. Just like other TACs, it is characterized by strictly unilateral pain in the trigeminal distribution, cranial autonomic features in the same area and agitation during exacerbations/attacks. It is different from other TACs in one aspect. While all other TACs are episodic, HC patients have continuous headaches with superimposed severe exacerbations. The central feature of HC is continuous background headache. However, the patients may be worried only for superimposed exacerbations. Focusing only on exacerbations and ignoring continuous background headache are the most important factors for the misdiagnosis of HC. A large number of patients may have migrainous features during exacerbation phase. Up to 70% patients may fulfill the diagnostic criteria for migraine during exacerbations. Besides migraine, its exacerbations can mimic a large number of other primary and secondary headaches. The other specific feature of HC is a remarkable response to indomethacin. However, a large number of patients develop side effects because of the long-term use of indomethacin. A few other medications may also be effective in a subset of patients with HC. Various surgical interventions have been suggested for patients who are intolerant to indomethacin. Several aspects of HC are still not defined. There is a great heterogeneity in types of patients or articles on the HC in the literature. Diagnostic criteria have been modified several times over the years. The current diagnostic criteria are too restrictive in some aspects. We suggest a more accommodating type of criteria for the appendix of International Classification of Headache Disorder (ICHD).
“…A response to indomethacin as 'sine qua non' for making a diagnosis of HC has been debated in the past (8,(27)(28)(29), and various authors suggested a possibility of indomethacin-resistant HC (7,8). HC-like headaches unresponsive to indomethacin were three times more common than typical HC in Marmura et al's case series.…”
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.
“…However, incidence and prevalence of indomethacin related side effects in patients of HC in other case series were about 20-50% [7][8][9]. Beside these, other cases of indomethacin related side effects in HC are mentioned in those conditions where drugs other than indomethacin were used.…”
Section: Discussionmentioning
confidence: 99%
“…We found at least 31 patients in the literature who showed complete and persistent effects by drugs other than indomethacin-cyclooxygenase-2 inhibitors, topiramate, piroxicam, melatonin, acetyl salicilic acid, ibuprofen, verapamil, and gabapentine (Table 1). Beside this, there are many case reports where above mentioned drugs and few other drugs (such as lamotrigine, methysegide, lithium carbonate, naproxen, paracetamol with caffeine) provided significant (although incomplete and/or transient) effect on the patients of HC [2,[6][7][8][9][10][15][16][17][18][19][20][21][22][23][24][25][26]. This observation is important.…”
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