Simultaneous occurrence of ipsilateral cluster headache and chronic paroxysmal hemicrania: a case report

A 42-year-old man came to our headache unit in October 1995 complaining of recurrent attacks of headache, which had begun in February 1991. Chronic cluster headache was diagnosed, and he was given verapamil, 360 mg per day. The attacks ceased in the following months and verapamil was stopped in March 1996. In May 1997, a recurrence of the attacks required the readministration of verapamil, 360 mg per day. The attacks decreased (one to three per week), but after 2 months the patient reported a worsening in his condition due to the appearance of shorter attacks, which were diagnosed as chronic paroxysmal hemicrania. The administration of indomethacin, 225 mg per day, resulted in the disappearance of the short attacks. The concomitant occurrence of attacks of cluster headache and chronic paroxysmal hemicrania suggests the presence of shared factors in the pathophysiology of the two forms of headache. This hypothesis is supported by previous reports in the literature.     

Chronic paroxysmal hemicrania: continued remission of symptoms after discontinuation of indomethacin

Discontinuation of indomethacin in chronic paroxysmal hemicrania (CPH) usually causes relapse of symptoms within a few days. A patient with characteristic symptoms of CPH is reported. The patient had daily attacks of unilateral headache accompanied by ipsilateral lacrimation and conjunctival injection, a frequency of attacks between four and fourteen per day, and never a headache-free day. He was treated with indomethacin, and all symptoms disappeared within 24 h. After three months indomethacin was discontinued. Six months after that the patient was still symptom-free.     

Hemicrania continua changed to chronic paroxysmal hemicrania after treatment with cyclooxygenase-2 inhibitor

Remission of hemicrania continua (HC) and transformation from HC to chronic paroxysmal hemicrania (CPH) are unusual. We report a patient with left-sided HC who, after a period of remission, presented as CPH. The continuous HC headache disappeared completely after initiating treatment with cyclooxygenase (COX)-2 inhibitor, but reappeared on the same side after 14 months remission with paroxysmal, frequent, intense and short-lasting headache attacks accompanied by ipsilateral cranial autonomic symptoms. This happened shortly after the treatment was discontinued because of withdrawal of the COX-2 inhibitor from the market. The response to indomethacin was prompt, and the patient became completely free from her paroxysmal headache with a dose of 50 mg 2 times daily. This case questions a possible modification effect on the course of HC by use of COX-2 inhibitor, as well as further supporting that some aspects of the pathophysiology of HC may resemble those of CPH, and may argue for common biological mechanisms in HC and CPH.     

Case Report: Chronic Cluster Headache with Unusual High Frequency of Attacks

SYNOPSIS
A case of chronic cluster headache with unusually frequent attacks (up to 30 per day) is discussed. Such numerous attacks are usually observed in chronic paroxysmal hemicrania only. The patient did not respond to indomethacin therapy, but rapid improvement during lithium treatment was observed.     

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.     

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.     

Prophylactic Treatment of Cluster Headache with Budipine

SYNOPSIS
For two years we have tested the prophylactic efficacy of budipine for the treatment of cluster headache. The open study was carried out on 27 men and 8 women. 23 patients suffered from episodic and 2 from chronic cluster headache. 7 patients had cluster-migraine, and 3 chronic paroxysmal hemicrania. 20 patients were free of cluster headache attacks 2–6 days after beginning daily prophylactic budipine therapy with 15–60 mg. 14 patients showed a markedly decreased frequency of attacks and the intensity of persisting attacks was diminished. One patient reported only a slight improvement. There were no non-responders to budipine therapy. 10 patients showed only mild budipine side effects. Budipine was found very effective for treatment of cluster headache.     

Trigeminal autonomic cephalalgias: current and future treatments

The trigeminal autonomic cephalgias include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). The evidence for the current treatment options for each of these syndromes is considered, including oxygen, sumatriptan, and verapamil in cluster headache, indomethacin in paroxysmal hemicrania, and intravenous lidocaine and lamotrigine in SUNCT. Some treatments such as topiramate have an effect in all of these, as well as in migraine and other pain syndromes. The involvement of the hypothalamus in functional imaging studies implies that this may be a substrate for targeting treatment options in the future.     

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: 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.     

An unusual form of TAC-TAC sine autonomic phenomena

The trigeminal autonomic cephalalgias (TACs) are characterized by the cardinal combination of short-lasting unilateral pain and autonomic phenomena affecting the head, including cluster headache, paroxysmal hemicrania and SUNCT. Infrequently reported have been cases with dissociation of these two cardinal features- usually with autonomic phenomena without pain, rather than the reverse. Herein is described the case of a 56-year-old man presenting with an unusual form of TAC resembling chronic paroxysmal hemicrania but with a lower frequency of occurrence, with temporal features not typical of previously described TACs, and consisting of severe unilateral head pain without any autonomic symptoms or signs, absolutely responsive to indomethacin.     

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.     

Chronic paroxysmal hemicrania: orbital phlebography and steroid treatment

A 62-year-old man with ankylosing spondylitis and with a 3-year history of chronic paroxysmal hemicrania is presented. Because of his ankylosing spondylitis naproxen was prescribed; this decreased the attacks of headache to about 50%. However, treatment with indomethacin and steroids eliminated the attacks completely, the former drug in 24 h but only when the drug was taken; the latter drug was completely effective after a week but with an effect that lasted half a year after the medication was stopped. Orbital phlebography showed changes similar to those previously observed in patients with Tolosa-Hunt syndrome and cluster headache. Venous vasculitis thus seems to be associated with all three disorders and may be a factor of etiologic significance.     

Chronic Paroxysmal Headache: Two Cases With Cerebrovascular Disease

Paroxysmal headaches often occur in benign headache disorders such as episodic cluster headache, chronic paroxysmal hemicrania (CPH) and episodic paroxysmal hemicrania. We report 2 patients with paroxysmal headaches occurring in association with cerebrovascular disease. The first patient had paroxysmal headaches from an arteriovenous malformation which resolved following embolization. In the second patient, headache followed a cerebral infarction and responded to treatment with indomethacin. We suggest that vascular disease may cause paroxysmal headaches resembling CPH. Patients with an atypical presentation of CPH warrant a neuroimaging procedure.     

Coexistence of cluster headache and paroxysmal hemicrania: does it exist? A case report and literature review

The coexistence of different types of trigeminal autonomic cephalalgias is a rare phenomenon. The two different types of headache may occur either at two different periods or simultaneously at the same time. We report a 22-year-old male who had cluster headache (CH) and chronic paroxysmal hemicrania (CPH) since the onset of symptoms. Both types of headache responded to indomethacin. Review of the literature suggests that simultaneous occurrence of CH and CPH in a patient may be both over and under reported.     

Unilateral photophobia or phonophobia in migraine compared with trigeminal autonomic cephalalgias

Our objective was to compare the presence of self-reported unilateral photophobia or phonophobia, or both, during headache attacks comparing patients with trigeminal autonomic cephalalgias (TACs)—including cluster headache, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and paroxysmal hemicrania—or hemicrania continua, and other headache types. We conducted a prospective study in patients attending a referral out-patient clinic over 5 months and those admitted for an intramuscular indomethacin test. Two hundred and six patients were included. In episodic migraine patients, two of 54 (4%) reported unilateral photophobia or phonophobia, or both. In chronic migraine patients, six of 48 (13%) complained of unilateral photophobia or phonophobia, or both, whereas none of the 24 patients with medication-overuse headache reported these unilateral symptoms, although these patients all had clinical symptoms suggesting the diagnosis of migraine. Only three of 22 patients (14%) suffering from new daily persistent headache (NDPH) experienced unilateral photophobia or phonophobia. In chronic cluster headache 10 of 21 patients (48%) had unilateral photophobia or phonophobia, or both, and this symptom appeared in four of five patients (80%) with episodic cluster headache. Unilateral photophobia or phonophobia, or both, were reported by six of 11 patients (55%) with hemicrania continua, five of nine (56%) with SUNCT, and four of six (67%) with chronic paroxysmal hemicrania. Unilateral phonophobia or photophobia, or both, are more frequent in TACs and hemicrania continua than in migraine and NDPH. The presence of these unilateral symptoms may be clinically useful in the differential diagnosis of primary headaches.     

Acetazolamide for the Treatment of Chronic Paroxysmal Hemicrania

SYNOPSIS
A 25-year-old patient presented with clinical characteristics of chronic paroxysmal hemicrania which failed to respond to indomethacin 300 mg daily. Total relief of headaches was obtained with acetazolamide 250 mg t.i.d.     

Hemicrania continua evolving from episodic paroxysmal hemicrania

A 45-year-old woman, who had been diagnosed in our unit with episodic paroxysmal hemicrania, was seen 2 years later for ipsilateral hemicrania continua in remitting form. Both types of headache had a complete response to indomethacin and did not occur simultaneously. The patient had a previous history of episodic moderate headaches that met criteria for probable migraine without aura and also had a family history of headache. The clinical course in this case suggests a pathogenic relationship between both types of primary headache.     

Chronic Paroxysmal Hemicrania: A Case Report

SYNOPSIS
Corneal indentation pulse amplitudes and intraocular pressures were obtained from a 51-year-old Danish woman-diagnosed as having chronic paroxysmal hemicrania. During headache attacks, values for both procedures were found to be markedly increased, especially on the symptomatic side. Complete relief of symptoms was obtained by indomethacin, prophylactically.     

Ocular Blood Flow Changes in Cluster Headache and Chronic Paroxysmal Hemicrania

Intraocular pressure and pulsatile ocular blood flow were recorded during and between attacks in patients suffering from cluster headache (n = 18) or chronic paroxysmal hemicrania (n = 7). Similarities, as well as significant differences, were observed between the two groups of patients, pointing to fundamental differences between the two disorders with regard to pathophysiology. Compared with healthy controls, the cluster headache patients demonstrated low pulsatile ocular blood flow values between attacks, with an increase to normal levels during pain. The chronic paroxysmal hemicrania patients, however, had normal values between attacks with a marked and significant increase to high values during attacks.