Abstract: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.
“…Tearing was the most common cranial autonomic feature (36%–77%) in all reported case series 7,11,14,25,28,29. Conjunctival injection, ptosis, nasal congestion and rhinorrhea are other common CAS in patients with HC.…”
Section: Clinical Featuresmentioning
confidence: 86%
“…In a series of 62 patients reported by Prakash et al,29 nine patients initially denied the presence of any CAS. However, CAS were noted later, on an objective assessment of the patients for CAS.…”
Section: Clinical Featuresmentioning
confidence: 96%
“…Approximately 50%–60% HC is chronic from the onset and 25%–35% HC evolves into continuous phase from the remitting form 12,14,25,26,29,34…”
Section: Classification and Variants Of Hcmentioning
confidence: 99%
“…It is usually started at a dose of 25 mg three times a day (tid). The drug is gradually titrated (25 mg tid every 3–5 days) up to 100 mg tid or until the patient gets complete relief 29. The dose required ranges from 25 to 500 mg/day 6,14.…”
Section: Managementmentioning
confidence: 99%
“…However, this part has not been studied much. There is just one case series that mentioned the time interval between administrations of indomethacin and a complete response 29. Only 10% patients showed a complete response within 24 hours.…”
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).
“…Tearing was the most common cranial autonomic feature (36%–77%) in all reported case series 7,11,14,25,28,29. Conjunctival injection, ptosis, nasal congestion and rhinorrhea are other common CAS in patients with HC.…”
Section: Clinical Featuresmentioning
confidence: 86%
“…In a series of 62 patients reported by Prakash et al,29 nine patients initially denied the presence of any CAS. However, CAS were noted later, on an objective assessment of the patients for CAS.…”
Section: Clinical Featuresmentioning
confidence: 96%
“…Approximately 50%–60% HC is chronic from the onset and 25%–35% HC evolves into continuous phase from the remitting form 12,14,25,26,29,34…”
Section: Classification and Variants Of Hcmentioning
confidence: 99%
“…It is usually started at a dose of 25 mg three times a day (tid). The drug is gradually titrated (25 mg tid every 3–5 days) up to 100 mg tid or until the patient gets complete relief 29. The dose required ranges from 25 to 500 mg/day 6,14.…”
Section: Managementmentioning
confidence: 99%
“…However, this part has not been studied much. There is just one case series that mentioned the time interval between administrations of indomethacin and a complete response 29. Only 10% patients showed a complete response within 24 hours.…”
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).
ImportanceTrigeminal autonomic cephalalgias (TACs) comprise a unique collection of primary headache disorders characterized by moderate or severe unilateral pain, localized in in the area of distribution of the first branch of the trigeminal nerve, accompanied by cranial autonomic symptoms and signs. Most TACs are rare diseases, which hampers the possibility of performing randomized clinical trials and large studies. Therefore, knowledge of treatment efficacy must be based only on observational studies, rare disease registries, and case reports, where real-world data and evidence play an important role in health care decisions.ObservationsChronic cluster headache is the most common of these disorders, and the literature offers some evidence from randomized clinical trials to support the use of pharmacologic and neurostimulation treatments. Galcanezumab, a monoclonal antibody targeting the calcitonin gene-related peptide, was not effective at 3 months in a randomized clinical trial but showed efficacy at 12 months in a large case series. For the other TACs (ie, paroxysmal hemicrania, hemicrania continua, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms), only case reports and case series are available to guide physicians in everyday management.Conclusions and RelevanceThe accumulation of epidemiologic, pathophysiologic, natural history knowledge, and data from case series and small controlled trials, especially over the past 20 years from investigators around the world, has added to the previously limited evidence and has helped advance and inform the treatment approach to rare TACs, which can be extremely challenging for clinicians.
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