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.     

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": 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". An indomethacin responsive headache. II. Autonomic function studies

Various autonomic parameters have been studied in two patients with "hemicrania continua", a newly described unilateral headache which is aborted by indomethacin. Striking findings were made on pupillometry: In both patients, isocoria was present when untreated. Bilateral instillation of tyramine in the conjunctival sac resulted in a late appearing anisocoria, with the smaller pupil on the symptomatic side. Indomethacin medication corrected this anomaly. These findings add further evidence to our firm belief that "hemicrania continua" differs fundamentally from chronic paroxysmal hemicrania, where such pupillometric changes are not found. There thus seem to be at least two different types of hemicranias with an absolute indomethacin effect.     

"Hemicrania continua": A third Norwegian case

Another case of hemicrania continua is described--that of a 54-year-old woman, who for the past 3 years has had continuous, left-sided headache. Before this period she had non-continuous headache, otherwise resembling the present one, for 3-4 years. In the chronic stage, the headache fluctuates considerably. Indomethacin in a dosage of 150 mg/day abolishes the headache completely. In hemicrania continua, there may thus, as in chronic paroxysmal hemicrania, be a non-chronic stage, antedating the chronic one. The relative importance of the two stages cannot be assessed at present.     

Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis

OBJECTIVE: To describe two cases of chronic paroxysmal hemicrania manifested by otalgia with a sensation of external acoustic meatus obstruction and to suggest that the trigeminal-autonomic reflex is a mechanism for the sensation of ear blockage. BACKGROUND: Maximum pain in chronic paroxysmal hemicrania is most often in the ocular, temporal, maxillary, and frontal regions. It is less often located in the nuchal, occipital, and retro-orbital areas. Review of the literature on chronic paroxysmal hemicrania found no reports of pain primarily localized to the ear and associated with a sensation of external acoustic meatus obstruction. METHODS: The history, physical examination, imaging studies, and successful treatment plan in two patients with otalgia and ear fullness and a subsequent diagnosis of chronic paroxysmal hemicrania are summarized. RESULTS: The first patient was a 42-year-old woman with a 10-year history of unilateral, severe, paroxysmal otalgia occurring five times a day with a duration of 2 to 60 minutes. During an attack, the ear became erythematous and the external acoustic meatus felt obstructed. There were no other associated autonomic signs. The second patient was a 49-year-old woman with a 3-year history of unilateral, severe, paroxysmal otalgia occurring 4 to 15 times a day with a duration of 3 to 10 minutes. During an attack, her ear felt obstructed, and she noted ipsilateral eyelid edema and ptosis. Both patients quickly became pain-free after taking indomethacin and required its continued use to prevent headache recurrence. CONCLUSIONS: Chronic paroxysmal hemicrania may be manifested by otalgia with a sensation of external ear obstruction. When the otalgia is paroxysmal, unilateral, severe, frequent, and associated with autonomic signs, one should consider the diagnosis of chronic paroxysmal hemicrania, especially because of the prompt response to indomethacin. The most important feature to consider when making the diagnosis of chronic paroxysmal hemicrania is the frequent periodicity of discrete, brief attacks of unilateral cephalgia separated by pain-free intervals. It is hypothesized that the sensation of ear obstruction in these patients is due to swelling of the external acoustic meatus mediated through increased blood flow by the trigeminal-autonomic reflex.     

Hemicrania continua: diagnostic criteria and nosologic status

Proposals for the diagnostic criteria for hemicrania continua (HC) and also for the nosological status of HC are set forth. The clinical constellation of symptoms and signs making up HC consists of: unilaterality without side shift; absolute indomethacin effect; and long-lasting repetitive attacks of varying duration, eventually with a chronic pattern, the pain being mild to severe. For the typical clinical picture of HC, including a positive 'indotest', we propose the term hemicrania continua vera. More or less analogous, but 'indotest-negative' clinical pictures have provisionally been termed hemicrania generis incerti (of undetermined nature). At the present level of knowledge, the diagnosis of hemicrania generis incerti should be made mostly by exclusion. HC may possibly best be classified along with chronic paroxysmal hemicrania (CPH) as this is the only other headache absolutely responsive to indomethacin. The bond between these two headaches on the one hand and cluster headache on the other should, at most, be a loose one. Interrelationships of these four classifiable headaches are briefly discussed.     

Episodic Paroxysmal Hemicrania: 3 New Cases and a Review of the Literature

SYNOPSIS
Episodic paroxysmal hemicrania (EPH) is a rare, benign disorder characterized by discrete bouts of hemicranial headaches separated by periods of pain-free remissions. EPH is frequently mistaken for episodic cluster headache because they have similar temporal profiles and clinical features. EPH is differentiated from cluster headaches by an increased frequency and a shorter duration of individual attacks. Establishing the diagnosis of EPH is important because of its unique response to treatment with indomethacin and not standard anti-cluster headache medications.     

Posttraumatic hemicrania continua

Hemicrania continua is a rare, benign headache disorder characterized by a low-level baseline hemicranial headache with superimposed exacerbations of more severe pain. Exacerbations last from minutes to days and may be associated with ipsilateral autonomic features such as ptosis, miosis, conjunctival injection, lacrimation, or rhinorrhea; when present, these features tend to be less pronounced than those seen with cluster headache. Response to treatment with indomethacin, in doses ranging from 25 to 300 mg per day, has been deemed a sine qua non of diagnosis. To date, in the majority of instances, hemicrania continua appears to have arisen de novo , without any identifiable trigger. We report four patients in whom the onset of hemicrania continua was temporally linked to head trauma.     

Chronic daily headache

Abstract Chronic daily headache relates to the daily or almost daily occurrence of headache in a nonparoxysmal pattern. In this review, I discuss the presentation, development, outcome, and treatment of chronic daily headache. In the context of the development of chronic daily headache, a headache continuum is presented along with its underlying pathophysiology. The treatment section covers rebound headache, analgesic and vasoconstrictor withdrawal, and the use of long-acting opioids in intractable patients. The review concludes with a discussion of hemicrania continua, an indomethacin-responsive headache syndrome.     

少见的三叉神经自主神经性头痛临床特点

目的:探讨伴有结膜充血及流泪的单侧短暂持续性神经痛样头痛(SUNCT)和阵发性偏侧头痛(PH)的临床特点.方法:回顾性分析2例SUNCT患者和3例PH患者的临床表现及治疗方法.结果:5例患者均表现为单侧眼眶、颞部的剧烈疼痛,并伴有同侧的颅内副交感神经激活症状,持续时间从20秒到30分钟,每天发作频率从8次到100次;S...     

Hemicrania continua with aura

Hemicrania continua is a primary headache disorder that is characterized by a continuous unilateral headache of moderate severity, exacerbations of severe pain and complete responsiveness to indomethacin. We report four patients with a unique variant of hemicrania continua: visual auras that precede or accompany the pain exacerbations.     

Headache following carotid endarterectomy: a prospective study

The occurrence of headache in the 28 days following surgery was studied in 50 consecutive patients (14F and 36M, mean aged 70 years) who underwent carotid endarterectomy for atheromatous carotid stenosis. Thirty-one patients (62%) reported headache. Headache occurred in the first five days after surgery in 87% of cases. Its characteristics and temporal profile were highly variable but it was mostly bilateral (74%), mild or moderate (78%), requiring no treatment (77%). No correlation was found between the occurrence of headache and degree of stenosis, intraoperative characteristics and past history of headache. In none of our patients was severe ipsilateral headache, cerebral hyperperfusion syndrome, or cluster-like hemicrania encountered and only five patients met the IHS criteria for post-endarterectomy headache. Post-endarterectomy headache is frequent when specifically looked for and is therefore not a single entity. The present IHS criteria are unsatisfactory and should be modified accordingly.     

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.     

Paroxysmale Hemikranie und SUNCT

Paroxysmal hemicrania is experienced as headache attacks with pain and accompanying symptoms similar to those of cluster headaches. Attacks are, however of shorter duration, occur more frequently, affect predominantly women and respond reliably to indomethacin. Paroxysmal hemicrania can also occur secondary to an identifiable cause. To exclude symptomatic, paroxysmal hemicrania, especially with an atypical clinical picture and poor response to indomethacin, a careful diagnostic approach is necessary. The SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is characterized by one-sided pain attacks of short duration, much shorter than other trigeminal autonomic cephalgias. Classically, the pain is accompanied by ipsilateral lacrimation and conjunctival injection. Some patients have been described with both cluster headache and trigeminal neuralgia. These patients should receive both diagnoses. It is important to differentiate these headache entities as specific therapy is needed for each to achieve optimal pain relief.     

Hemicrania Continua-like headache due to nonmetastatic lung cancer--a vagal cephalalgia

A 72-year-old man presented with a 7-week history of a new onset constant severe right-sided headache associated with redness and tearing of the right eye, which resolved on indomethacin due to nonmetastatic small cell carcinoma producing a large suprahilar mass. This is the first case report of a hemicrania continua-like headache with autonomic features due to lung cancer. I propose the term "vagal cephalalgia" to include headache and/or facial pain due to nonmetastatic lung cancer and cardiac cephalalgia which result from vagal afferent stimulation.     

Chronic daily bilateral headache responsive to indomethacin

Indomethacin is known to be specifically effective for chronic paroxysmal hemicrania, episodic paroxysmal hemicrania, and hemicrania continua. Different forms of idiopathic stabbing headaches have also been responsive to indomethacin, but less consistently than the others. Two cases of indomethacin-responsive headache are reported. One patient presented with what appeared to be new-onset, chronic, daily, bilateral headache aggravated by coughing. Both the chronic daily headache and the exacerbations induced by coughing were suppressed with indomethacin therapy. The second patient experienced hemicrania continua responsive to indomethacin, and the response persisted even when the headache evolved into bilateral continuous pain. There may be other idiopathic primary headache disorders that are peculiarly responsive to indomethacin. When any primary headache disorder does not respond to standard therapy, a brief therapeutic trial of indomethacin is warranted.     

Hemicrania Continua–Like Headache With Leprosy: Casual or Causal Association?

Hemicrania continua is a strictly unilateral, moderate to severe, continuous, indomethacin-responsive primary headache disorder with ipsilateral autonomic cranial symptoms at the time of exacerbations. We describe a 30-year-old woman with a 4-month history of indomethacin-responsive hemicrania continua–like headache and one-month history of mononeuritis multiplex due to leprosy. Indomethacin was successfully weaned off after completion of antileprotic therapy.