Headache & Pain
Primary headache associated with sexual activity
Nov. 30, 2024
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Paroxysmal hemicrania …
- Updated 01.17.2024
- Released 09.02.1994
- Expires For CME 01.17.2027
Paroxysmal hemicrania
Introduction
Overview
The author reviews paroxysmal hemicrania, one of the trigeminal autonomic cephalalgias that is indomethacin-responsive (130; 30). Strictly unilateral pain of short attack length (typically 20 minutes), high frequency of attacks (often 10 per day), and associated lateralized cranial autonomic features (such as lacrimation, conjunctival injection, and nasal symptoms) should trigger the diagnosis to be considered. When paroxysmal hemicrania is considered, investigation for pituitary gland pathology with dedicated MRI and blood tests is recommended, based on large case series. Paroxysmal hemicrania is absolutely responsive to indomethacin, when it is tolerated, at doses from 25 to 275 mg daily.
Key points
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• Paroxysmal hemicrania is a unilateral, severe, short-lasting headache, typically of 20 minutes duration and occurring approximately 10 times a day. | |
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• Paroxysmal hemicrania attacks are associated with cranial autonomic symptoms, such as lacrimation, conjunctival infection, and nasal symptoms. | |
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• Paroxysmal hemicrania responds absolutely to indomethacin given orally. | |
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• It is recommended that any patient diagnosed with paroxysmal hemicrania undergo investigation by MRI and blood tests for pituitary gland dysfunction. |
Historical note and terminology
In 1974, Sjaastad and Dale reported what they described rather aptly as a new treatable headache entity (111). They subsequently coined the term “chronic paroxysmal hemicrania” to describe these patients (112). Later, a remitting form of the disease was recognized and termed “episodic hemicrania continua” (57; 11; 83; 43; 126). The release of the second edition of the International Headache Society Classification resulted in the introduction of the umbrella terminology “paroxysmal hemicrania,” which is recognized to have both an episodic and a chronic form (49).
Clinical manifestations
Presentation and course
Paroxysmal hemicrania is characterized by multiple, brief, intense, daily focal attacks of head pain. The pain is unilateral and always affects the same side. The pain is usually most severe in the auriculotemporal area, the forehead, and above or behind the ear, although it can occur anywhere in the head. It may spread to involve the ipsilateral shoulder, arm, and neck. The pain is described as excruciating, throbbing, boring, or pulsating. Between attacks, the patient may have tenderness in the symptomatic area (22). During attacks, the patient usually sits quietly or may "curl up in bed," which contrasts with the usually increased restlessness and activity during cluster headache attacks (41). Some patients have interparoxysmal pain, so the differential diagnosis with hemicrania continua can be problematic as both are indomethacin-sensitive (21). This may be the basis for a reported transition from hemicrania continua to paroxysmal hemicrania in a single case report (80).
Paroxysmal hemicrania usually begins in adulthood. The mean age is 34 years, ranging from 2 to 81 years. Paroxysmal hemicrania has been reported in children (55; 16; 39; 76; 120; 127; 122; 95; 59; 52; 35; 81), and their symptoms are very much the same as those in adults (10; 38). It has been reported, and it is the author’s experience, that the condition settles with age (72). As in adults, the differential diagnosis of cluster headache can be troublesome (115). The author has seen a number of children between 4 and 12 years of age with typical indomethacin responses. In the author’s experience, the disorder settles in this age group after 2 to 3 years, and one patient has been reported with that behavior (121). The attack profile of paroxysmal hemicrania is highly characteristic. The frequency of attacks ranges from 2 to 30 per 24-hour period (108). Attacks usually last between 2 and 25 minutes and occasionally as long as 60 minutes. In a prospective study of 105 attacks, the mean duration was 13 minutes, with a range of 3 to 46 minutes, a mean attack frequency of 14 in a 24-hour period (range 4 to 38), and even circadian distribution (97). Nocturnal attacks associated with the REM phase of sleep have also been described (54).
Ipsilateral lacrimation is reported to occur in about 80% of attacks. When the lacrimation is bilateral, it is predominantly on the side of the pain (05). Conjunctival injection on the symptomatic side occurs during two thirds of attacks (22). Some patients have ipsilateral eyelid edema and slight miosis. Photophobia may accompany some attacks, and increased forehead sweating, especially on the symptomatic side, is observed in a few patients. Photophobia, when present, is often unilateral to the side of the pain (51). Although not widely recognized, a sense of aural fullness is reported in paroxysmal hemicrania (22) and may account for some patients reported not to have cranial autonomic features (92).
Three cases are documented of nonlateralized, short-lasting headache that responded to indomethacin, but these patients did not have cranial autonomic symptoms (91; 79; 09); one might consider this a different syndrome--perhaps paroxysmal cephalalgia (70). Cases of short-lasting presentation with bilateral cranial autonomic features responding to indomethacin have also been reported (33; 28), as has a case with unilateral pain without apparent cranial autonomic features (61). In the latter case, the description of the relevant negatives is incomplete, and the nosology of these entities is unclear.
Although most attacks are sudden and spontaneous, approximately 10% may be precipitated mechanically, either by bending or rotating the head (05). Attacks may also be provoked by external pressure against the transverse processes of C2, C3, C4, or the greater occipital nerve (113). The relationship between menstruation and paroxysmal hemicrania attacks is undetermined (63; 62). Birth control pills do not seem to influence the attack frequency, and menopause has no reported effect. Unusual presentation with otalgia and a feeling of fullness in the ear has been reported (15), as has extratrigeminal pain (31). A report from the Mayo Clinic suggests that the largest part of the phenotype can be seen without a response to indomethacin (13). Cases of mixed trigeminal autonomic cephalalgias have been reported and underline the premise of ICHD-3 that there is a shared pathophysiology (123).
The features of the International Classification of Headache Disorders (ICHD) third edition beta criteria are listed below (49).
Paroxysmal hemicrania has two key forms. The episodic form occurs during a period lasting 7 days to 1 year and is separated by pain-free periods that last 1 month or more. The chronic form has attacks that occur for more than 1 year without remission or with remissions lasting less than 1 month.
To be diagnosed with paroxysmal hemicrania, patients must experience each of the following:
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A. At least 20 attacks fulfilling criteria B through E | ||
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B. Severe, unilateral orbital, supraorbital, or temporal pain that lasts 2 to 30 minutes | ||
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C. Headache associated with at least one of the following symptoms or signs, ipsilateral to the pain: | ||
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- Conjunctival injection and/or lacrimation | ||
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D. Attack frequency of more than five headaches daily for more than one half of the time | ||
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E. Attacks are prevented absolutely by therapeutic doses of indomethacin | ||
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F. Not better accounted for by another ICHD-3 diagnosis | ||
The criteria are very much like ICHD-2 (48), with the notable standardization of the cranial autonomic features amongst the trigeminal autonomic cephalgias. The sense of aural fullness, which is common in trigeminal autonomic cephalalgias (41), has been removed in ICHD-3, which this author sees as a mistake to be corrected in ICHD-4.
Prognosis and complications
There are no systematic data on the prognosis of paroxysmal hemicrania. Sjaastad followed a patient for 45 years, during which time the patient was on indomethacin for 33 years. The drug never stopped working, and the problem seemed to become less troublesome with time (106).
No secondary complications exist except those that arise as a result of long-term indomethacin therapy, such as gastritis, gastric ulcers, and gastrointestinal bleeding.
Biological basis
Etiology and pathogenesis
The etiology of paroxysmal hemicrania is unknown; however, given its similarities with cluster headache (73), a disorder of the CNS seems the most likely explanation. Head or neck trauma is reported by approximately 20% of patients (05), although these findings do not differ significantly from cluster headache or migraine (56; 105).
The pathophysiological mechanisms responsible for pain in paroxysmal hemicrania are unknown. As with other primary headaches, it seems likely that there is a hereditary element, and a family with paroxysmal hemicrania has been reported (25). The pain is strictly unilateral and appears to be in the distribution of the ipsilateral trigeminal nerve. The pain and the cranial autonomic symptoms, such as lacrimation and conjunctival injection, have linked the disorder to cluster headache under the classification of trigeminal autonomic cephalalgia (43). It is likely to be a brain disorder, as is cluster headache (40).
Dynamic tonometric studies have shown that in chronic paroxysmal hemicrania significant attack-related increases in corneal indentation pulse amplitudes, ocular blood flow, and intraocular pressure occur in paroxysmal hemicrania (112; 98). These findings probably result from acute vasodilatation resulting in increased intraocular volume due to a neurogenic impulse and are mediated by the release of vasoactive neuropeptides (42). Similar to cluster headache, calcitonin gene-related peptide and vasoactive intestinal polypeptide are released in the cranial circulation during acute attacks of chronic paroxysmal hemicrania (42; 32). POEMS can have a paroxysmal hemicrania-like presentation (88), again implicating peptide mechanisms.
Increased tearing, conjunctival injection, and nasal secretion are probably due to cranial parasympathetic activation. Attack-related heart rhythm disturbances have been observed in a few patients. These cardiac abnormalities include bradycardia and sinoatrial block, bundle-branch block with episodes of atrial fibrillation, and multiple extrasystoles (111; 90; 97). This may indicate dysfunction in the central control of the autonomic nervous system during chronic paroxysmal hemicrania attacks that affect both sympathetic and parasympathetic systems. A lesion in the pericarotid area inside the cavernous sinus where trigeminal, sympathetic, and parasympathetic fibers come together has been advanced to explain the pain and cranial autonomic changes (78); however, this fails to explain the paroxysmal nature of the pain attacks (43). There is no cognitive deficit in paroxysmal hemicrania (34).
An important finding for paroxysmal hemicrania is activation on PET in the posterior hypothalamic region (67), as seen in other trigeminal autonomic cephalalgias (23) and in the ventral midbrain, as seen in hemicrania continua (68). A case of a patient in the arctic region with less troublesome attacks in the light season reinforces the possibility of hypothalamic mechanisms in this disorder (08). The latter may be a link to indomethacin sensitivity, and the whole picture reinforces the unique nature of this condition.
The mechanism behind the absolute responsiveness to indomethacin is now starting to be unraveled. There is a clear effect on nitric oxide synthase of indomethacin that other NSAIDS, such as ibuprofen and naproxen, do not have (117; 118). Interestingly, although an animal model of TAC pathophysiology demonstrates that superior salivatory nucleus-evoked lacrimation can be attenuated by indomethacin (01), trigeminally evoked parasympathetic activation in humans is unaffected by indomethacin (75). It appears to be independent of indomethacin’s effect on prostaglandin synthesis, as other nonsteroidal anti-inflammatory agents have little effect (107) or no (86; 03) effect on chronic paroxysmal hemicrania. Sjaastad’s first case (personal communication) and cases this author has seen respond well to aspirin.
Epidemiology
Paroxysmal hemicrania is a rare syndrome, although with increasing awareness of it, it is perhaps being recognized more frequently. The prevalence of paroxysmal hemicrania is not known, although an important contribution from the Vaga study of headache epidemiology was to identify one possible patient with the syndrome among 1838 adults aged 18 to 65 who were quizzed (110). The data are important because the authors also reported data for cluster headache, 0.3% of the cohort (109), and saw two patients with SUNCT and one with hemicrania continua in the same group (110). Remarkably, this suggests that SUNCT is more common than paroxysmal hemicrania and that paroxysmal hemicrania is much less common than cluster headache. This is certainly consistent with this author’s referral practice.
Chronic paroxysmal hemicrania was originally reported in females, although males can be affected (94; 96). In a 1989 survey of 84 reported cases, 59 were females and 25 males, with a female-to-male ratio of 2.36 to 1 (05). In the largest single-case series, the male-to-female ratio was equal (22).
Prevention
No method of preventing paroxysmal hemicranias has been established. Once the diagnosis is made, indomethacin will prevent the attacks; but when indomethacin is discontinued, symptoms usually reappear within 12 hours to a few days (03).
Differential diagnosis
Confusing conditions
The differential diagnosis for paroxysmal hemicrania is between other trigeminal autonomic cephalalgias (43; 103) and secondary forms of chronic paroxysmal hemicrania. Mistaking chronic paroxysmal hemicrania for cluster headache is problematic because, generally speaking, treatments for cluster headache are not effective in chronic paroxysmal hemicrania. There is a case report of the two conditions existing together (19), and there have been claims that cluster headache can respond to indomethacin (93). These cases need to be objectively determined with blinded indomethacin tests. Chronic paroxysmal hemicrania has been said to be more often seen in females, although the largest case series does not bear this out (22). Given the effect of indomethacin is relatively rapid in onset (03), it could be advocated that all patients diagnosed with trigeminal autonomic cephalgias who do not have a contraindication could have a short course of indomethacin at the start of treatment to detect the indomethacin-sensitive group, at least until a reliable biological marker becomes available. Whether indomethacin-insensitive paroxysmal hemicrania exists is moot (13; 36), and more research is needed. A comparison found no particular clinical difference between paroxysmal hemicrania and cluster headache (132), and the editorial commentary (12) is reminiscent of Sjaastad’s original remark that indomethacin “hits the nail on the head” in paroxysmal hemicrania. The only trial of indomethacin in cluster headache suggests it does not work (02), but published placebo-controlled experience is lacking. Episodic paroxysmal hemicrania is differentiated by periods in which there are no attacks, and the considerations for indomethacin are similar.
Associated or underlying disorders
The coexistence of chronic paroxysmal hemicrania with trigeminal neuralgia, or chronic paroxysmal hemicrania-tic syndrome, is recognized (45). It is important to recognize because both chronic paroxysmal hemicrania and trigeminal neuralgia require treatment (18; 46; 65; 133). Acetazolamide has been suggested as an addition to this therapeutic armamentarium (100). In line with the proposed changes to the International Headache Society classification and respecting the fact that both episodic and chronic paroxysmal hemicrania are reported (14), the term “paroxysmal hemicrania-tic” is preferable (44). Chronic paroxysmal hemicrania may coexist with benign cough headache, and both syndromes respond to indomethacin (66).
Secondary paroxysmal hemicrania is well described, and investigations are required to identify or exclude treatable underlying causes (124). Secondary paroxysmal hemicrania should be considered if the clinical picture is typical, but treatment response is poor or if there are neurologic signs. Paroxysmal hemicrania may be seen after head trauma and even with typical migraine aura where comorbidity with migraine is a reasonable explanation (69; 102; 89). A reasonably complete screen of a patient with chronic paroxysmal hemicrania, considering the associated clinical problems reported, would include a blood count looking for thrombocythemia (60); erythrocyte sedimentation rate and vasculitic investigations (74); a brain-imaging procedure looking for an intracranial tumor, such as a lesion in the region of the sella turcica (37; 128; 101) or orbit (20); cerebral metastases (64) or elsewhere (74); or a brain hemorrhage (07). It is important to exclude pituitary pathology by imaging and blood tests (58; 131; 06). Other structural mimics of chronic paroxysmal hemicrania include an arteriovenous malformation (84), anterior clinoid meningioma (26), cavernous sinus meningioma, or the condition has been reported after carotid aneurysm embolization (50). A secondary form of the disorder has been reported with the use of phosphodiesterase inhibitors (119). Secondary chronic paroxysmal hemicrania is more likely if the patient requires high doses (greater than 200 mg/day) of indomethacin (114), although this author has seen a case in which a patient took 400 mg daily, and extensive investigations over the years have yielded nothing. Should the pain become bilateral, a lumbar puncture should be carried out to look for intracranial hypertension, even in the face of a response to indomethacin (45). When appropriate, an electrocardiogram and Holter monitor should be considered to look for bundle branch block or atrial fibrillation (97), and a chest x-ray should be considered to look for a Pancoast tumor (29).
Diagnostic workup
A good clinical history, a detailed neurologic examination, and a therapeutic trial of indomethacin are necessary to diagnose paroxysmal hemicrania. It is generally agreed that an MRI brain with pituitary views is appropriate at baseline.
Management
The treatment of chronic paroxysmal hemicrania is prophylactic (41). Indomethacin is the drug of choice, and its effect on symptoms is absolute. When a patient with a unilateral headache has a relatively high attack frequency (more than four attacks in 24 hours), a drug trial with indomethacin should be considered. Trial doses should be increased to at least 150 mg per 24 hours for 3 or 4 days, with a maximum dose of 275 mg daily. The beneficial effect is seen within a few hours to 5 days. The maintenance dosage is usually 25 to 100 mg/day but varies among individuals, and the response persists for years (85). When indomethacin is discontinued, symptoms usually reappear within 12 hours to a few days (03). To prevent gastric complications, long-term indomethacin therapy may be combined with antacids and histamine blockers or other gastric mucosa protective agents. Nausea, vomiting, vertigo, and purpura have been reported during indomethacin use. There has been some controversy about whether sumatriptan is effective (87) or ineffective (27; 04) in chronic paroxysmal hemicrania. The issue is unresolved, but it seems possible that it is a matter of attack length. The COX-2 inhibitors celecoxib (71), valdecoxib (withdrawn from the United States and other markets due to adverse effects) (104), and rofecoxib (withdrawn from United States and other markets due to adverse effects) (Goadsby unpublished observations) may be useful in paroxysmal hemicrania. This author and others have had useful responses from topiramate (24; 17). As with cluster headache, pterygopalatine ganglion blockade has been reported to be useful (77). Neuromodulation with noninvasive vagus nerve stimulation has been reported to be useful (125; 53), and its possible mechanisms have been reviewed (129).
Outcomes
Patients who respond to indomethacin will continue to do so indefinitely.
Special considerations
Pregnancy
In several patients with chronic paroxysmal hemicrania, attacks have disappeared or improved during pregnancy and returned after delivery (108; 116), although this author has managed a female with otherwise typical disease who had attacks throughout pregnancy. There is no risk to the mother or child if chronic paroxysmal hemicrania occurs during pregnancy. It is not advisable to continue indomethacin. No specific treatment is available during pregnancy for this condition, and its management is extremely difficult.
Anesthesia
There is no contraindication to the use of general or local anesthetics in patients undergoing surgical procedures.
References
- 01
- Akerman S, Holland PR, Summ O, Lasalandra MP, Goadsby PJ. A translational in vivo model of trigeminal autonomic cephalalgias: therapeutic characterization. Brain 2012;135(Pt 12):3664-75. PMID 23065481
- 02
- Antonaci F, Costa A, Ghirmai S, Sances G, Sjaastad O, Nappi G. Parenteral indomethacin (the INDOTEST) in cluster headache. Cephalalgia 2003;23:193-6. PMID 12662186
- 03
- Antonaci F, Pareja JA, Caminero AB, Sjaastad O. Chronic paroxysmal hemicrania and hemicrania continua. Parenteral indomethacin: the 'Indotest.' Headache 1998a;38:122-8. PMID 9529768
- 04
- Antonaci F, Pareja JA, Caminero AB, Sjaastad O. Chronic paroxysmal hemicrania and hemicrania continua: lack of efficacy of sumatriptan. Headache 1998b;38:197-200. PMID 9563210
- 05
- Antonaci F, Sjaastad O. Chronic paroxysmal hemicrania (CPH): a review of the clinical manifestations. Headache 1989;29:648-56. PMID 2693408
- 06
- Arkink EB, Schmitz N, Schoonman GG, et al. The anterior hypothalamus in cluster headache. Cephalalgia 2017;37(11):1039-50. PMID 27530226
- 07
- Bazan R, de Almeida LA, da Rocha FC, Raffin CN, Fonseca RG. Headache secondary to haemorrhagic stroke resembling paroxysmal hemicrania. Arq Neuropsiquiatr 2008;66:761-2. PMID 18949281
- 08
- Bekkelund SI, Lilleng H. Impact of extreme light exposure during summer season in an arctic area on patients with migraine and chronic paroxysmal hemicrania (CPH). Cephalalgia 2006;26(9):1153-6. PMID 16919069
- 09
- Bingel U, Weiller C. An unusual indomethacin sensitive headache: a case of bilateral episodic paroxysmal hemicrania without autonomic symptoms. Cephalalgia 2005;25:148-50. PMID 15658952
- 10
- Blankenburg M, Hechler T, Dubbel G, Wamsler C, Zernikow B. Paroxysmal hemicrania in children--symptoms, diagnostic criteria, therapy and outcome. Cephalalgia 2009;29(8):873-82. PMID 19236385
- 11
- Blau JN, Engel H. Episodic paroxysmal hemicrania: a further case and review of the literature. J Neurol Neurosurg Psychiatry 1990;53:343-4. PMID 2187950
- 12
- Boes C. Differentiating paroxysmal hemicrania from cluster headache. Cephalalgia 2005;25:241-3. PMID 15773820
- 13
- Boes CJ, Dodick DW. Refining the clinical spectrum of chronic paroxysmal hemicrania: a review of 74 patients. Headache 2002;42:699-708. PMID 12390632
- 14
- Boes CJ, Matharu MS, Goadsby PJ. The paroxysmal hemicrania-tic syndrome. Cephalalgia 2003;23(1):24-8. PMID 12534576
- 15
- Boes CJ, Swanson JW, Dodick DW. Chronic paroxysmal hemicrania presenting as otalgia with a sensation of external acoustic meatus obstruction: two cases and a pathophysiologic hypothesis. Headache 1998;38:787-91. PMID 11279905
- 16
- Broeske D, Lenn NJ, Cantos E. Chronic paroxysmal hemicrania in a young child: possible relation to ipsilateral occipital infarction. J Child Neurol 1993;8:235-6. PMID 8409264
- 17
- Camarda C, Camarda R, Monastero R. Chronic paroxysmal hemicrania and hemicrania continua responding to topiramate: two case reports. Clin Neurol Neurosurg 2008;110:88-91. PMID 17954009
- 18
- Caminero AB, Pareja JA, Dobato JL. Chronic paroxysmal hemicrania-tic syndrome. Cephalalgia 1998;18:159-61. PMID 9595211
- 19
- Centonze V, Bassi A, Causarano V, Dalfino L. Simultaneous occurrence of ipsilateral cluster headache and chronic paroxysmal hemicrania: a case report. Headache 2000;40:54-6. PMID 10759906
- 20
- Choi HA, Lee MJ, Chung CS. Chronic paroxysmal headache secondary to an orbital metastatic leiomyosarcoma: a case report. Cephalalgia 2018;38(2):389-92. PMID 28058942
- 21
- Cittadini E, Goadsby PJ. Hemicrania continua: a clinical study of 39 patients with diagnostic implications. Brain 2010;133(Pt 7):1973-86. PMID 20558416
- 22
- Cittadini E, Matharu MS, Goadsby PJ. Paroxysmal hemicrania: a prospective clinical study of thirty-one cases. Brain 2008;131:1142-55.** PMID 18252775
- 23
- Cohen AS, Goadsby PJ. Functional neuroimaging of primary headache disorders. Curr Neurol Neurosci Rep 2004;4:105-10. PMID 14984681
- 24
- Cohen AS, Goadsby PJ. Paroxysmal hemicrania responding to topiramate. J Neurol Neurosurg Psychiatr 2007;78:96-7. PMID 17172571
- 25
- Cohen AS, Matharu MS, Goadsby PJ. Paroxysmal hemicrania in a family. Cephalalgia 2006;26:486-8. PMID 16556252
- 26
- Dafer RM, Hocker S, Kumar R, McGee J, Jay WM. Resolution of paroxysmal hemicrania after resection of intracranial meningioma. Semin Ophthalmol 2010;25(1-2):34-5. PMID 20148710
- 27
- Dahlof C. Subcutaneous sumatriptan does not abort attacks of chronic paroxysmal hemicrania (CPH). Headache 1993;33:201-2. PMID 8388371
- 28
- Degirmenci Y, Kececi H. Can paroxysmal hemicrania be bilateral? A case report. Neurol Sci 2016;37(8):1377-8. PMID 27023075
- 29
- Delreux V, Kevers L, Callewaert A. Hemicranie paroxystique inaugurant un syndrome de Pancoast. Revue Neurologique 1989;145:151-2. PMID 2727538
- 30
- Diener HC, Tassorelli C, Dodick DW. Management of trigeminal autonomic cephalalgias including chronic cluster: a review. JAMA Neurol 2023;80:308-19. PMID 36648786
- 31
- Dodick DW. Extratrigeminal episodic paroxysmal hemicrania. Further clinical evidence of functionally relevant brain stem connections. Headache 1998;38:794-8. PMID 11279907
- 32
- Edvinsson L, Ekman R, Goadsby PJ. Measurement of vasoactive neuropeptides in biological materials: problems and pitfalls from 30 years of experience and novel future approaches. Cephalalgia 2010;30(6):761-6. PMID 20925142
- 33
- Evans RW. Bilateral paroxysmal hemicrania with autonomic symptoms: the first case report. Cephalalgia 2008;28:191-2. PMID 17941880
- 34
- Evers S, Bauer B, Suhr B, Voss H, Frese A, Husstedt IW. Cognitive processing is involved in cluster headache but not in chronic paroxysmal hemicrania. Neurology 1999;53:357-63. PMID 10430426
- 35
- Evers S, Summ O, Frese A. Very young age of onset in trigemino-autonomic cephalalgias - case report and review of the literature. Cephalalgia 2020;40(12):1385-8. PMID 32664738
- 36
- Fuad F, Jones NS. Paroxysmal hemicrania and cluster headache: two discrete entities or is there an overlap. Clin Otolaryngol 2002;27:472-9. PMID 25588019
- 37
- Gawel MJ, Rothbart P. Chronic paroxysmal hemicrania which appears to arise from either third ventricle pathology or internal carotid artery pathology. Cephalalgia 1992;12:327. PMID 25358210
- 38
- Ghosh A, Varghese L, Burish MJ, Szperka CL. Trigeminal autonomic cephalalgias and neuralgias in children and adolescents: a narrative review. Curr Neurol Neurosci Rep 2023;23:539-49. PMID 37572226
- 39
- Gladstein J, Holden EW, Peralta L. Chronic paroxysmal hemicrania in a child. Headache 1994;34:519-20. PMID 8002324
- 40
- Goadsby PJ. Pathophysiology of cluster headache: a trigeminal autonomic cephalgia. Lancet Neurology 2002;1:37-43. PMID 12849458
- 41
- Goadsby PJ. Trigeminal autonomic cephalalgias. Continuum (Minneap Minn) 2012;18(4):883-95. PMID 22868548
- 42
- Goadsby PJ, Edvinsson L. Neuropeptide changes in a case of chronic paroxysmal hemicrania--evidence for trigemino-parasympathetic activation. Cephalalgia 1996;16:448-50. PMID 8902256
- 43
- Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic features, including new cases. Brain 1997;120:193-209. PMID 9055807
- 44
- Goadsby PJ, Lipton RB. Paroxysmal hemicrania-tic syndrome. Headache 2001;41:608-9.** PMID 11437905
- 45
- Hannerz J. Trigeminal neuralgia with chronic paroxysmal hemicrania: the CPH-tic syndrome. Cephalalgia 1993;13:361-4. PMID 8242731
- 46
- Hannerz J. The second case of chronic paroxysmal hemicrania-tic syndrome [Editorial Comment]. Cephalalgia 1998;18:124.
- 47
- Hannerz J, Jogestrand T. Intracranial hypertension and sumatriptan efficacy in a case of chronic paroxysmal hemicrania which became bilateral. (The mechanism of indomethacin in CPH). Headache 1993;33:320-3. PMID 8394301
- 48
- Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders. 2nd edition. Cephalalgia 2004;24 Suppl 1:1-160.
- 49
- Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders, 3rd edition. Cephalalgia 2018;38(1):1-211. PMID 29368949
- 50
- Irimia P, Barbosa C, Martinez-Vila E. Paroxysmal hemicrania after carotid aneurysm embolization. Cephalalgia 2005;25:1096-8. PMID 16232164
- 51
- Irimia P, Cittadini E, Paemeleire K, Cohen AS, Goadsby PJ. Unilateral photophobia or phonophobia in migraine compared with trigeminal autonomic cephalalgias. Cephalalgia 2008;28:626-30. PMID 18422722
- 52
- Ishii R, Fukazawa R, Takezawa H, Fujii A. Case report: shortest course of pediatric paroxysmal hemicrania. Headache 2019;59(7):1084-7. PMID 31264201
- 53
- Kamourieh S, Lagrata S, Matharu MS. Non-invasive vagus nerve stimulation is beneficial in chronic paroxysmal hemicrania. J Neurol Neurosurg Psychiatry 2019;90(9):1072-4. PMID 30709897
- 54
- Kayed K, Godtlibsen OB, Sjaastad O. Chronic paroxysmal hemicrania. 4. "REM sleep locked" nocturnal headache attacks. Sleep 1978;1:91-5. PMID 227025
- 55
- Kudrow DB, Kudrow L. Successful aspirin prophylaxis in a child with chronic paroxysmal hemicrania. Headache 1989;29:280-1. PMID 2745065
- 56
- Kudrow L. Cluster headache: Mechanisms and Management. Oxford: Oxford University Press, 1980.
- 57
- Kudrow L, Esperanca P, Vijayan N. Episodic paroxysmal hemicrania. Cephalalgia 1987;7:197-201. PMID 3652203
- 58
- Levy M, Matharu MS, Meeran K, Powell M, Goadsby PJ. The clinical characteristics of headache in patients with pituitary tumours. Brain 2005;128:1921-30.** PMID 15888539
- 59
- Mack KJ, Goadsby PJ. Trigeminal autonomic cephalalgias in children and adolescents: cluster headache and related conditions. Semin Pediatr Neurol 2016;23(1):23-6. PMID 27017018
- 60
- MacMillan JC, Nukada H. Chronic paroxysmal hemicrania. N Z Med J 1989;102:251-2. PMID 2726045
- 61
- Maggioni F. Episodic paroxysmal hemicrania without autonomic symptoms: are there possible subgroups in PH. Cephalalgia 2010;30(4):504-6. PMID 19515120
- 62
- Maggioni F. Further on menstrual paroxysmal hemicrania. Cephalalgia 2011;31(5):637. PMID 21278240
- 63
- Maggioni F, Palmieri A, Viaro F, Mainardi F, Zanchin G. Menstrual paroxysmal hemicrania, a possible new entity. Cephalalgia 2007;27(9):1085-7. PMID 17655719
- 64
- Mariano HS, Bigal ME, Bordini CA, Speciali JG. Chronic paroxysmal hemicrania (CPH)-like syndrome as a first manifestation of cerebral metastasis of parotid epidermoid carcinoma: a case report. Cephalalgia 1999;19(4):442.
- 65
- Martinez-Salio A, Porta-Etessam J, Perez-Martinez D, Balseiro J, Gutierrez-Rivas E. Chronic paroxysmal hemicrania-tic syndrome. Headache 2000;40:682-5. PMID 10971666
- 66
- Mateo I, Pascual J. Coexistence of chronic paroxysmal hemicrania and benign cough headache. Headache 1999;39(6):437-8. PMID 11279922
- 67
- Matharu MS, Cohen AS, Frackowiak RS, Goadsby PJ. Posterior hypothalamic activation in paroxysmal hemicrania. Ann Neurol 2006;59:535-45.** PMID 16489610
- 68
- Matharu MS, Cohen AS, McGonigle DJ, Ward N, Frackowiak RS, Goadsby PJ. Posterior hypothalamic and brainstem activation in hemicrania continua. Headache 2004;44:747-61. PMID 15330820
- 69
- Matharu MS, Goadsby PJ. Post-traumatic chronic paroxysmal hemicrania (CPH) with aura. Neurology 2001;56:273-5. PMID 11160973
- 70
- Matharu MS, Goadsby PJ. Bilateral paroxysmal hemicrania or bilateral paroxysmal cephalalgia, another novel indomethacin-responsive primary headache syndrome. Cephalalgia 2005;25:79-81. PMID 15658943
- 71
- Mathew NT, Kailasam J, Fischer A. Responsiveness to celecoxib in chronic paroxysmal hemicrania. Neurology 2000;55:316. PMID 10908918
- 72
- Mauritz MD, Enninger A, Wamsler C, Wager J, Zernikow B. Long-term outcome of indomethacin treatment in pediatric patients with paroxysmal hemicrania-a case series. Children (Basel) 2021;8(2). PMID 33546261
- 73
- May A, Bahra A, Buchel C, Frackowiak RS, Goadsby PJ. Hypothalamic activation in cluster headache attacks. Lancet 1998;351:275-8. PMID 9690407
- 74
- Medina JL. Organic headaches mimicking chronic paroxysmal hemicrania. Headache 1992;32:73-4. PMID 1551789
- 75
- Moller M, Schroder C, Iwersen-Bergmann S, Mehnert J, May A. Indomethacin has no effect on trigeminally provoked parasympathetic output. Cephalalgia 2022;42(1):37-43. PMID 34407645
- 76
- Moorjani BI, Rothner AD. Indomethacin-responsive headaches in children and adolescents. Semin Pediatr Neurol 2001;8:40-5. PMID 11332865
- 77
- Morelli N, Mancuso M, Felisati G, et al. Does sphenopalatine endoscopic ganglion block have an effect in paroxysmal hemicrania. A case report. Cephalalgia 2010;30(3):365-7. PMID 19438918
- 78
- Moskowitz MA. Basic mechanisms in vascular headache. Neurol Clin 1990;8:801-15. PMID 2175382
- 79
- Mulder LJ, Spierings EL. Non-lateralized pain in a case of chronic paroxysmal hemicrania. Cephalalgia 2004;24(1):52-3. PMID 14687013
- 80
- Muller KI, Bekkelund SI. Hemicrania continua changed to chronic paroxysmal hemicrania after treatment with cyclooxygenase-2 inhibitor. Headache 2011;51:300-5. PMID 21284612
- 81
- Myers KA, Barmherzig R, Raj NR, et al. The spectrum of indomethacin-responsive headaches in children and adolescents. Cephalalgia 2022;42(8):793-7. PMID 35302385
- 82
- Nesbitt AD, Goadsby PJ. Cluster Headache. BMJ 2012;344(2012):e2407. PMID 22496300
- 83
- Newman LC, Gordon ML, Lipton RB, Kanner R, Solomon S. Episodic paroxysmal hemicrania: two new cases and a literature review. Neurology 1992a;42:964-6.** PMID 1579250
- 84
- Newman LC, Herskovitz S, Lipton R, Solomon S. Chronic paroxysmal headache: two cases with cerebrovascular disease. Headache 1992b;32:75-6. PMID 1551790
- 85
- Pareja J, Caminero AB, Franco E, Casado JL, Pascual J, Sanchez del Rio M. Dose, efficacy and tolerability of long-term indomethacin treatment of chronic paroxysmal hemicrania and hemicrania continua. Cephalalgia 2001;21:869-78.** PMID 11903285
- 86
- Pareja J, Sjaastad O. Chronic paroxysmal hemicrania and hemicrania continua. Interval between indomethacin administration and response. Headache 1996;36:20-3. PMID 8666531
- 87
- Pascual J, Quijano J. A case of chronic paroxysmal hemicrania responding to subcutaneous sumatriptan. J Neurol Neurosurg Psychiatry 1998;65(3):407. PMID 9728969
- 88
- Paviour DC, Ellis CM. Paroxysmal hemicrania and POEMS syndrome: further evidence that neuropeptides are implicated in primary headache. Cephalalgia 2008;28:1204-6. PMID 18644027
- 89
- Peng KP, Schellong M, May A. Aura in trigeminal autonomic cephalalgia is probably mediated by comorbid migraine with aura. Cephalalgia 2022;42:31-6. PMID 34407649
- 90
- Petty RG, Rose FC. Chronic paroxysmal hemicrania: first reported British case. BMJ 1983;286:483. PMID 6401553
- 91
- Pollmann W, Pfaffenrath V. Chronic paroxysmal hemicrania: the first possible bilateral case. Cephalalgia 1986;6:55-7. PMID 3698094
- 92
- Prakash S, Belani P, Susvirkar A, Trivedi A, Ahuja S, Patel A. Paroxysmal hemicrania: a retrospective study of a consecutive series of 22 patients and a critical analysis of the diagnostic criteria. J Headache Pain 2013;14(1):26. PMID 23566235
- 93
- Prakash S, Shah ND, Chavda BV. Cluster headache responsive to indomethacin: case reports and a critical review of the literature. Cephalalgia 2010;30(8):975-82. PMID 20656709
- 94
- Price RW, Posner JB. Chronic paroxysmal hemicrania: a disabling headache syndrome responding to indomethacin. Ann Neurol 1978;3:183-4. PMID 655667
- 95
- Raieli V, Cicala V, Vanadia F. Pediatric paroxysmal hemicrania: a case report and some clinical considerations. Neurol Sci 2015;36(12):2295-6. PMID 26265294
- 96
- Rapoport AM, Sheftell FD, Baskin SM. Chronic paroxysmal hemicrania: case report of the second known definite occurrence in a male. Cephalalgia 1981;1:67-70. PMID 7346175
- 97
- Russell D. Chronic paroxysmal hemicrania: severity, duration and time of occurrence of attacks. Cephalalgia 1984;4:53-6. PMID 6538816
- 98
- Russell D, Christoffersen B, Hoerven I. Chronic paroxysmal hemicrania: case report. Headache 1978;18:99-100. PMID 649387
- 99
- Russell D, Storstein L. Chronic paroxysmal hemicrania: heart rate changes and ECG rhythm disturbances. A computerized analysis of 24h ambulatory ECG recordings. Cephalalgia 1984;4:135-44. PMID 6733781
- 100
- Sanahuja J, Vazquez P, Falguera M. Paroxysmal hemicrania-tic syndrome responsive to acetazolamide. Cephalalgia 2005;25:547-9. PMID 15955044
- 101
- Sarov M, Valade D, Jublanc C, Ducros A. Chronic paroxysmal hemicrania in a patient with a macroprolactinoma. Cephalalgia 2006;26:738-41. PMID 16686914
- 102
- Seidel S, Wober C. Paroxysmal hemicrania with visual aura in a 17-year-old boy. Headache 2009;49(4):607-9. PMID 19348039
- 103
- Silberstein SD, Lipton RB, Goadsby PJ. Headache in Clinical Practice, 1st ed. Oxford: ISIS Medical Media, 1998.
- 104
- Siow HC. Seasonal episodic paroxysmal hemicrania responding to cyclooxygenase-2 inhibitors. Cephalalgia 2004;24:414-5. PMID 15096232
- 105
- Sjaastad O. Cluster Headache Syndrome. London: W.B. Saunders, 1992.
- 106
- Sjaastad O. Chronic paroxysmal hemicrania, hemicrania continua and SUNCT: the fate of the three first described cases. J Headache Pain 2006;7(3):151-6.** PMID 16817054
- 107
- Sjaastad O, Antonaci F. A piroxicam derivative partly effective in chronic paroxysmal hemicrania and hemicrania continua. Headache 1995;35(9):549-50. PMID 8530280
- 108
- Sjaastad O, Apfelbaum R, Caskey W, et al. Chronic paroxysmal hemicrania (CPH). The clinical manifestations. A review. Ups J Med Sci Suppl 1980;31:27-33. PMID 6935856
- 109
- Sjaastad O, Bakketeig LS. Cluster headache prevalence. Vaga study of headache epidemiology. Cephalalgia 2003;23(7):528-33. PMID 12950378
- 110
- Sjaastad O, Bakketeig LS. The rare, unilateral headaches. Vaga study of headache epidemiology. J Headache Pain 2007;8(1):19-27. PMID 17221345
- 111
- Sjaastad O, Dale I. Evidence for a new, treatable headache entity. Headache 1974;14:105-8. PMID 4851935
- 112
- Sjaastad O, Horven I, Vennerod AM. A new headache syndrome. Headache resembling cluster headache (Horton's headache), with recurring bouts of homolateral retrobulbar neuritis, partial factor XII deficiency, bleeding tendency and a heterolateral convulsive episode. Headache 1976;16(1):4-10. PMID 1254470
- 113
- Sjaastad O, Russell D, Saunte C, Horven I. Chronic paroxysmal hemicrania VI. Precipitation of attacks. Further studies on the precipitation mechanism. Cephalalgia 1982;2:211-4. PMID 7159922
- 114
- Sjaastad O, Stovner LJ, Stolt-Nielsen A, Antonaci F, Fredriksen TA. CPH and hemicrania continua: requirements of high dose indomethacin dosages- an ominous sign. Headache 1995;35:363-7. PMID 7635724
- 115
- Solomon S, Newman LC. Chronic paroxysmal hemicrania in a child. Headache 1995;35:234. PMID 7775183
- 116
- Stein HJ, Rogado AZ. Chronic paroxysmal hemicrania--two new patients. Headache 1980;20:72-6. PMID 7372474
- 117
- Summ O, Andreou AP, Akerman S, Goadsby PJ. A potential nitrergic mechanism of action for indomethacin, but not of other COX inhibitors--relevance to indomethacin-sensitive headaches. J Headache Pain 2010;11:477-83. PMID 20978816
- 118
- Summ O, Andreou AP, Akerman S, Holland PR, Hoffmann J, Goadsby PJ. Differential actions of indomethacin: clinical relevance in headache. Pain 2021;162:591-95. PMID: 32796319.** PMID 32796319
- 119
- Taga A, Russo M, Genovese A, Manzoni GC, Torelli P. Paroxysmal hemicrania-like headache secondary to phosphodiesterase inhibitors administration: a case report. Headache 2017;57(4):663-4. PMID 28188618
- 120
- Talvik I, Koch K, Kolk A, Talvik T. Chronic paroxysmal hemicrania in a 3-year, 10-month-old female. Pediatr Neurol 2006;34:225-7. PMID 16504793
- 121
- Talvik I, Peet A, Talvik T. Three-year follow-up of a girl with chronic paroxysmal hemicrania. Pediatr Neurol 2009;40:68-9. PMID 19068259
- 122
- Tarantino S, Vollono C, Capuano A, Vigevano F, Valeriani M. Chronic paroxysmal hemicrania in paediatric age: report of two cases. J Headache Pain 2011;12:263-7. PMID 21340658
- 123
- Totzeck A, Diener HC, Gaul C. Concomitant occurrence of different trigeminal autonomic cephalalgias: a case series and review of the literature. Cephalalgia 2014;34(3):231-5. PMID 24065715
- 124
- Trucco M, Mainardi F, Maggioni F, Badino R, Zanchin G. Chronic paroxysmal hemicrania, hemicrania continua and SUNCT syndrome in association with other pathologies: a review. Cephalalgia 2004;24:173-84. PMID 15009010
- 125
- Tso AR, Marin J, Goadsby PJ. Noninvasive vagus nerve stimulation for treatment of indomethacin-sensitive headaches. JAMA Neurol 2017;74(10):1266-7.** PMID 28846758
- 126
- Veloso GG, Kaup AO, Peres MF, Zukerman E. Episodic paroxysmal hemicrania with seasonal variation: case report and the EPH-cluster headache continuum hypothesis. Arq Neuropsiquiatr 2001;59:944-7. PMID 11733843
- 127
- Vieira JP, Salgueiro AB, Alfaro M. Short-lasting headaches in children. Cephalalgia 2006;26(10):1220-4. PMID 16961790
- 128
- Vijayan N. Symptomatic chronic paroxysmal hemicrania. Cephalalgia 1992;12:111-3. PMID 1290485
- 129
- Villar-Martinez MD, Goadsby PJ. Non-invasive neuromodulation of the cervical vagus nerve in rarer primary headaches. Frontiers in Pain Research 2023;4:1062892. PMID 36994091
- 130
- Villar-Martinez MD, Moreno-Ajona D, Chan C, Goadsby PJ. Indomethacin-responsive headaches- a narrative review. Headache 2021;61:700-14.** PMID 34105154
- 131
- Wilbrink LA, Ferrari MD, Kruit MC, Haan J. Neuroimaging in trigeminal autonomic cephalgias: when, how, and of what. Curr Opin Neurol 2009;22(3):247-53.** PMID 19434790
- 132
- Zidverc-Trajkovic J, Pavlovic A, Mijajlovic M, Jovanovic Z, Sternic N, Kostic V. Cluster headache and paroxysmal hemicrania: differential diagnosis. Cephalalgia 2005;25:244-8. PMID 15773821
- 133
- Zukerman E, Peres MFP, Kaup AO, Monzillo PH, Costa AR. Chronic paroxysmal hemicrania-tic syndrome. Neurology 2000;54:1524-6. PMID 10751271
Contributors
All contributors' financial relationships have been reviewed and mitigated to ensure that this and every other article is free from commercial bias.
Author
-
Peter J Goadsby MD PhD FRS
Dr. Goadsby of King’s College London received consulting fees from Abbvie, Aeon Biopharma, Amgen, Eli Lilly, Epalex, Lundbeck, Novartis, Pfizer, Sanofi, Satsuma, Shiratronics, and Teva Neurosciences and a grant from Celegene.
See Profile
Editor
-
Shuu-Jiun Wang MD
Dr. Wang of the Brain Research Center, National Yang-Ming University, and the Neurological Institute, Taipei Veterans General Hospital, has no relevant financial relationships to disclose.
See Profile
Former Authors
- Ninan Mathew MD (original author)
Patient Profile
- Age range of presentation
-
- 6 to 65+ years
- Sex preponderance
-
- female>male, >2:1
- Heredity
-
- none
- Population groups selectively affected
-
- none selectively affected
- Occupation groups selectively affected
-
- none selectively affected
ICD & OMIM codes
- ICD-9
-
- Cluster headache: 346.2
- ICD-10
-
- Chronic paroxysmal hemicrania: G44.0
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