Hemicrania Continua: A Case Responsive to Piroxicam-Beta-Cyclodextrin

The case of a 53-year-old woman is described, who since the age of 38 years had been suffering from a unilateral headache that was at first remitting, but then evolved into an unremitting course. The headache was burning in quality, located in the left forehead and face, and was accompanied by mild ipsilateral autonomic symptoms. The clinical picture, as well as the absolute response to indomethacin, suggested a diagnosis of hemicrania continua. The presence of mild gastric discomfort on continuous indomethacin administration made us transfer the patient to another NSAID (piroxicam-beta-cyclodextrin). With this drug, complete relief and good tolerability was obtained.     

"Hemicrania Continua": A Clinical Review

Hemicrania continua (HC) is a headache entity completely responsive to indomethacin. Since 1984, 18 cases have been described, 15 females and 3 males, i.e. a F:M ratio of 5.0. The finding of a female preponderance, like that in chronic paroxysmal hemicrania, is a new observation. HC is, in general, a unilateral headache in the sense that it sets in on one side and subsequently sticks to this side. In two cases, both sides might possibly be involved, when the pain was at its maximum. In another (somewhat dubious) case the headache was bilateral. The pain was continuous from the beginning in 8 of 18 cases (early stage ratio continuous: non-continuous = 0.8). Over time, the headache developed a continuous character in 16 of the 18 cases, producing a "continuous: non-continuous ratio" of 8:1. The intensity of pain generally was moderate and was not reported as excruciatingly severe by any patient. The autonomic involvement from a clinical point of view, was clearly less pronounced than that of other unilateral headaches, such as cluster headache and chronic paroxysmal hemicrania.     

Hemicrania Continua Responsive to Intravenous Methyl Prednisolone

Hemicrania continua (HC) is a strictly unilateral, continuous primary headache disorder with periodic exacerbations, usually accompanied by cranial autonomic disturbances. Exquisite and persistent effect of indomethacin is a fundamental property of HC. We report 2 patients of HC in which attacks were successfully eliminated by repeated infusion of methyl prednisolone.     

Hemicrania Continua: Remitting Stage Evolved From the Chronic Form

It is known that in hemicrania continua the chronic stage may be preceded by a remitting stage. In the present communication, the reverse sequence has been demonstrated, ie, a transition from the chronic to the remitting stage. The headache was characterized by a chronic pattern from the beginning until the commencement of indomethacin treatment, which provided long-lasting relief. Withdrawal of the drug resulted in reappearance of headaches with the same clinical features but in a remitting fashion. Resumption of indomethacin treatment had a dramatic effect on the remitting headaches. Such a temporal evolution of symptoms is consistent with the prevailing view that in a patient with hemicrania continua, there may be two temporal patterns.     

Hemicrania Continua and Symptomatic Medication Overuse

SYNOPSIS
Hemicrania continua (HC) is a rare, strictly unilateral, non-paroxysmal headache disorder characterized by its absolute responsiveness to indomethacin. The pain is usually moderate in intensity and frequently associated with a superimposed"jabs and jolts" headache.
We report two cases of HC which presented as chronic daily headache (CDH) with abortive medication overuse.
CDH can be due to transformed migraine (TM), new daily persistent headache (NDPH), chronic tension-type headache, and HC. All can be unilateral, and all can be associated with medication overuse. Our two cases meet the criteria for HC based on indomethacin responsiveness. One meets the criteria for TM, the other NDPH. Is HC a distinct disorder, or a subset of these other disorders? CDH with medication overuse includes in its differential diagnosis HC.     

Hemicrania Continua in a Black Patient the Importance of the Non-Continuous Stage

A 52-year-old black female for 19 years had severe intermittent unilateral headaches that demonstrated the "clustering" phenomenon. She was initially diagnosed as having episodic cluster headache. Response to lithium carbonate, ergotamine and courses of corticosteroids was, however, only partial. In December 1989 the headache pattern changed and she developed severe unilateral hemicranial headache that was continuous and non-remitting. This responded immediately and persistently to oral indomethacin. A diagnosis of hemicrania continua (HC) was made. The initial intermittent headache syndrome appears to have been the pre-continuous stage of hemicrania continua, and not episodic cluster headache as previously supposed. The pre-continuous phase of hemicrania continua may thus masquerade as episodic cluster headache by reason of its intermittency and "clustering". In this case, the intermittent stage was protracted. This stage may, conceivably, even be a permanent one. To our knowledge, this is the first report of hemicrania continua in a black African.     

Chronic Paroxysmal Hemicrania and Hemicrania Continua. Interval Between Indomethacin Administration and Response

The interval between indomethacin dosage and clinical response was assessed in hemicrania continua (n=12) and chronic paroxysmal hemicrania (n=11) sufferers. The number of trials per patient ranged from I to 30. At the time of testing, the patients had "considerable" pain after discontinuation of the drug. The dosage used was the usual one for that patient at the given pain level; ie, 25 or 50 mg tid. All the patients had a complete, long-term response to treatment. Nevertheless, the average interval between drug intake and pain relief during the present study ranged beween 30 minutes and 48 hours in both disorders. In most patients (10 in both groups), the indomethacin effect was complete within 24 hours, and frequently within 8 hours. It is suggested that interindividual differences in dosage and timing to abolish the headaches may be due to different bioavailability or individual sensitivity. Recommendations on indomethacin testing in unilateral headaches are given.     

Coexisting Trigeminal Autonomic Cephalalgias and Hemicrania Continua

The trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC) share many clinical characteristics including unilateral pain and ipsilateral autonomic features. We report a patient with a history of migraine without aura who developed cluster headache and HC simultaneously. The distinctive clinical features and differential response profiles to various treatments indicates that they are distinct disorders. We then review previous reports of patients with coexisting TACs and HC and discuss the relationship between these families of primary headache disorders.     

CPH and Hemicrania Continua: Requirements of High Indomethacin Dosages -An Ominous Sign?

Two female patients, one with chronic paroxysmal hemi-crania and one with hemicrania continua, had a continuously high requirement of indomethacin, ie, ³ 225 mg per day, for 4 and 7 years, respectively. In the hemicrania continua patient, a right (symptomatic side) C7 root affection due to disc herniation was demonstrated. Removal of the disc relieved the arm pain completely, and reduced the head pain and indomethacin requirement considerably initially. The other patient suffered from the unremitting form of chronic paroxysmal hemicrania with right-sided attacks from the age of 16. Indomethacin, 200 to 250 mg per day generally kept the headache at bay, but during exacerbations, especially during menstrual periods, the dosage transitorily had to be increased to 250 to 350 mg per day. ACT scan with contrast at aged 18 (1987) was negative. In 1992, she started having new symptoms, including numbness on the ipsilateral side of the face and arm and difficulty swallowing. An MR scan showed a meningioma originating in the roof of the cavernous sinus on the symptomatic side. The meningioma was surgically removed. The postoperative indomethacin requirement was reduced, but only transiently. Patients with chronic paroxysmal hemicrania (CPH) and hemicrania continua (HC) with a continuously high indomethacin requirement may have grave additional disorders and should consequently be followed closely.     

Hemicrania Continua‐Like Headache Related to Transdermal Nitroglycerine Therapy

Several cases of symptomatic hemicrania continua (HC) have been reported. A 66‐year‐old man, suffering from migraine without aura, presented with a four month history of a new headache fulfilling the ICHD 3beta clinical criteria for HC. HC onset was strictly related to the use of transdermal nitroglycerine patch (TNP). In agreement with the cardiologist, TNP was discontinued and the headache promptly disappeared; symptoms reappeared within 6‐12 hours after nitroglycerine reintroduction. After permanent discontinuation of TNP, headache disappeared at one year follow‐up. To the best of our knowledge, this is the first report of the occurrence of an HC‐like headache related to TNP.     

Remitting Form of Hemicrania Continua With Seasonal Pattern

Hemicrania continua is a primary headache syndrome characterized by a continuous, unilateral headache that is completely responsive to indomethacin. Hemicrania continua exists in continuous and remitting forms. Ten cases of the remitting form have been reported, none of which have had a seasonal pattern. We report a patient with remitting hemicrania continua with a clear seasonal predilection.     

Diagnostic Delay and Suboptimal Management in a Referral Population With Hemicrania Continua

Aim of the Study.— To investigate a clinical population of patients with hemicrania continua (HC), looking at the diagnostic problems they have encountered and their use of healthcare resources and at issues relating to the effectiveness of treatments.
Materials and Methods.— We directly interviewed 25 patients fulfilling the International Classification of Headache Disorders, 2nd edition diagnostic criteria for HC selected among 1612 subjects attending the INI Grottaferrata Headache Clinic over a 3-year period.
Results.— No patient had received a correct diagnosis before being seen at our headache clinic. In total, 85% of the patients consulted a physician within 5 months of the onset of the symptoms but mean time to diagnosis was 5 years (SD 4.9). The average number of physicians seen before the condition was properly diagnosed was 4.6 (SD 2.2). General practitioners (100%), neurologists (80%), ear, nose, and throat surgeons (44%), ophthalmologists (40%), and dentists (32%) were the physicians most commonly consulted. All the patients had previously received an incorrect diagnosis. Migraine (52%), CH (28%), sinus headache (20%), and dental pain (20%) were the most common wrong diagnoses reported. Some 36.0% of patients had undergone ineffective invasive treatments. The patients had tried, on average, 3.6 (SD 2.1) classes of drugs. Nonsteroidal anti-inflammatory drugs (NSAIDs) (92%), triptans (32%), antidepressants (32%), and antiepileptics (24%) were the most commonly used. Patients rated 73.7% of medications as ineffective, 22.5% (all NSAIDs) as partially effective, and 3.7% (rofecoxib and nimesulide) as effective.
Conclusions.— Hemicrania continua may be misdiagnosed and mistreated even by neurologists. There is a need for greater awareness and understanding of this condition.     

Hemicrania Continua. Unquestionably a Trigeminal Autonomic Cephalalgia

Hemicrania continua (HC) is a well‐known primary headache. The present version of the International Classification of Headache Disorders lists HC in the “other primary headaches” group. However, evidence has emerged demonstrating that HC is a phenotype that belongs to the trigeminal autonomic cephalalgias together with cluster headache, paroxysmal hemicrania (PH), and short‐lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing. This is supported by a common general clinical picture – paroxysmal, fluctuating, unilateral, side‐locked headaches located to the ocular, frontal, and/or temporal regions, accompanied by ipsilateral autonomic dysfunctions including for example, tearing and conjunctival injection. Apart from the remarkable clinical similarities, the absolute and incomparable effect of indomethacin in HC parallels the effect of this drug in PH, suggesting a shared core pathogenesis. Finally, neuroimage findings demonstrate a posterior hypothalamic activation in HC similarly to cluster headache, PH, and short‐lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing. Taken together, data indicate that HC is certainly a type of trigeminal autonomic cephalalgia that should no longer be placed in a group of miscellaneous primary headache disorders.     

Can Indomethacin Act as a Disease Modifying Agent in Hemicrania Continua? A Supportive Clinical Case

The natural history of hemicrania continua (HC) is not well-known. Most sufferers have the unremitting form and thus may have a lifetime duration of pain. There are only very rare cases in the literature documenting HC remission after treatment of the unremitting form with indomethacin. As the prolonged use of indomethacin can cause tremendous medical morbidity, knowledge of its true action on the course of HC is essential. The question arises: Does indomethacin have disease-modifying capacities for HC or does it just suppress the pain without altering the underlying disease pathogenesis? A case patient is presented who had indomethacin-responsive unremitting HC and after a period of daily use of indomethacin she was able to reduce her dose to one single 25-mg tablet 3 days per week and still remain pain free, suggesting disease modification. The case patient's history will be further discussed as well as the consideration that in some instances indomethacin can alter the natural history of HC.