Chronic Paroxysmal Hemicrania Presenting As Toothache

Chronic Paroxysmal Hemicrania Presenting as Toothache

Roben E, Delcanho. BDSc, MS Chronic paroxysmal hemicrania is an intermittent head-pain problem that is characterized by pain paroxysms lasting about IS min- Steven B, Graff-Radford, DDS tites- The attacks usually produce pain in the frontotemporal region Section of Orofaciai Pain and are responsive to indomethacin. A set of symptoms that defines UCLA, Schooi of Dentistry chronic paroxysmal hemicrania is presented, and two cases in 10833 Le Conte Avenue which the presenting symptom was toothache are reported. It is Los Angeles, Califomis 90024 emphasized that clinicians should consider chronic paroxysmal Correspondence to Dr Delcanho hemicrania in the differential diagnosis of orofaciai pain. J OROFACIAL PAIN 1993;7:300-306,

ost pain problems perceived in the teeth can be adequate- ly managed by common dental techniques. However, Mpatients may present with pain in the orofaciai region that does not arise in the teeth but rather is referred from other organ systems. Such prohlems require spectahzed managetiient, not mechanical dental interventions. To formulate a differential dtag- nosis when a patient presents with orofaciai pain, the clinician must be familiar with all possible pain conditions that may be perceived in the orofaciai region. Most of these conditions have been previously discussed in the dental literature,' ' Chronic paroxysmal hemicrania (CPH), a complex set of symptoms first described by Sjaastad and Dale," may present in the orofaciai area but has thus far not heen reviewed in the dental literature. This article briefly reviews this syndrome and presents two case reports in which CPH initially presented as maxillary tooth pain.

Clinical Features

Chronic paroxysrnal hemicrania is a rare, yet well-character i zed,* chronic unilateral headache disorder, with specific inclusionary criteria as determined by the International Headache Society.^ Clinically similar to cluster headache, but with important differ- ences, the main features of CPH are: 1. Unilateral, intense, throhbtng periorbital/temporal pain 2. Multiple attacks per day 3. Attacks of short duration 4. Chronic daily pattern of attacks, without remission 5. Female preponderance 6- Associated autonomie symptoms 7. Attacks that are mechanically precipitated by neck movements in some patients 8. Absolute respotise to indomethacin

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Age and Gender Another study' of 84 CPH patients found the Age of onset of CPH is variable, with Antonaci mean number of attacks to be 10.8 ± 5,0 over a and Sjaastad' finding a range of 11 to 8 I years and 24-hour period. These multiple attacks are a prin- a mean age of onset of 33.7 years. This is very sim- cipal characteristic that helps differentiate CPH ilar to the age of onset in cluster headache patients from cluster headache patients, who aver- patients. age only 1 to 3 attacks per day (mean 1.67) during In an early CPH review,' eight of the definite the cluster periods.' diagnoses and seven of the ten possible diagnoses The number of pain attacks may be cyclic, witb described were made for women. This was the first periods of increased and decreased attack frequen- remarkable difference noted when comparing the cy. These periods of relative exacerbation and pain problem to tbat in Ekbom et AVS classic clus- remission blend into each otber over weeks and ter headache study,' in which 143 patients were months. Similarly, there is variation in the severity male and only 20 were female. As more cases came of the pain attacks, with transitions from mild to to light, it became apparent tbat CPH primarily intense attack periods occurring gradually over a affects women, although there are definite exam- few days. When low-grade pain occurs there may ples of men with CPH. be difficulty in discerning the temporal pattern of The relationship of CPH to tbe female reproduc- the pain. However, unlike cluster headache, clear- tive cycle has been studied because of this gender cut pain remission phases do not occur in CPH, difference. In 10 pregnant CPH patients, a trend although up to 42% of patients enter a remission toward reduction of or freedom from headache phase in which the pain appears in more episodic was observed, but symptoms resumed immediately clusters, rather than in the regular daily pattern," after parturition." The pain pattern may resemble tbat of both cluster Chronic paroxysmal hemicrania has also been and migraine headaches during this phase. known to begin after pregnancy. Tbe administration of birth control pills has been sbown to bave Duration of Attacks no influence on tbe tendency of attacks, while The pain attacks of CPFl generally do not iasc as menstruation has been shown to have botb posi- long as those of cluster headache. Russell' has tive and negative effects on CPH attacks, Tbere is found the mean duration to be 13,3 minutes with insufficient evidence of the influence of menopause a range of 3 to 46 minutes. By comparison, the to draw any conclusions at tbis time. same study reported cluster attacks to have a mean duration of 45 minutes. More recently, Antonaci Location and Character of the Pain and Sjaastad" have reported CPH attacks generally lasting 10 to 30 minutes (mean 20,9 minutes) with Typically, GPH patients complain of recurrent a range of 5 to 45 minutes. daily attacks of intense, throbbing, periorbital and/or temporal pain. These attacks of severe pain are unilateral, always occur on the same side, and Timing of Attacks run a chronic course without remission. The pain The attacks may appear at any particular time, IS usually located in the oculotemporal or temporal sometimes with clockwork regularity. Russell's region, but it may involve the entire hemicranium, study' of CPH patients found that attacks seemed including the jaws, and so may be confused with to occur mostly in the late afternoon or during the other pain syndromes found in this region (eg, evening, with less than one third of CPH attacks toothache]. Patients with CPH have been known occurring during sleep. It seems therefore that noc- to have root canal therapy and even extraction of turnal pain is not as strong a feature in CPH as in teeth in an attempt to eliminate what was felt to be cluster headaches, in which the pain characteristi- the source of the pain.' The pain has also been cally awakens a patient from sleep. reported to spread down through the neck, to the ipsilateral shoulder and arm. Associated Symptoms Autonomie symptoms are similar to, but generally Frequency of Attacks milder tban, those found in cluster headache. Such In one study,' attack frequency in CPH patients symptoms may include nasal congestion, lacrima- has been found to vary between 4 and 38 occur- tion, conjunctival injection, rhinorrhea, ptosis, and rences over a 24-bour period (mean of 13.6), eyelid edema on the symptomatic side. Nausea and

Journal of Orofacial Pain 301 De lean ho

vomiting, which accompany many other headache tion tbat had any benefit prior to ii^<-' "f types, are not seen. Sweating, another ciinicai indometbacin. In both patients, the initial response characteristic seen on the symptomatic side in clus- to indomethacin {75 mg per day) occurred within ter headache, has heen studied in six female CPH tbe first 24 bours. Sometimes tbe patients benefit patients using an evaporimeter.'" The results show at smaller dose levels, and as little as 12.5 mg per only two of the six patients to have increased day can be effective. Tbe maximum required dose sweating on the symptomatic side, and it was appears to be 250 mg. Tbe effects of jndometbacin therefote concluded that the sweating pattern seen bave been reported to continue for several years in cluster headache is not a characteristic of CPH. afcer the initiation of treatment witbout the devel- Interestingly, in patients whose attacks could be opment of tolerance. Discontinuation of the precipitated mechanically, marked forehead sweat- indomethacin usually causes return of symptoms ing on the symptomatic side was strongly predic- within 2 days. Jensen et al'" have repotted one tive that a pain attack was about to ensue. patient who, after 3 months of indomethacin treatment, stopped the medication and remained pain free after 6 months. Precipitating Factors Studies comparing various other anti-inflamma- Mechanically precipitated attacks have been tory medications found that none were any more described in detail by Sjaastad et al.""" Clinically effective than acetylsalicylic acid or as effective as the same as spontaneous attacks, these mechanical JndomethacJn. attacks were triggered by either flexion movement of the head and neck or by pressure applied to ten- der cervical areas. The head flexion-triggered attacks could only be induced if the head itself, Laboratory Findings and not the entire body, was hent. The pain occurred from 5 seconds to 1 minute after the stimulus, depending on the state of the individual The following significant findings were made via patient. Rotating the head maximally to the symp- laboratory investigation. The interested reader is tomatic side could also precipitate such attacks. referred to Sjaastad'" for a more complete discus- Following neck flexjon in susceptible patients, tbe sion of CPH, including the various tests and stud- autonomie phenomena appeared many seconds ies thus far carried out on CPH patients. ahead of the pain, thus indicating clearly that such phenomena are not due to tfie pain per se." Corneal Indentation Pulse CCIP) Amplitudes Alternatively, pressure to certain cervical trigger spots could immediately precipitate attacks of The CIP amplitudes of CPH patients were found CPH, with the pain and autonomie phenomena to he at the upper range of normal between appearing together. The mechanisms hehind attacks; however, the CIP amplitudes were signifi- mechanical precipitation of CPH remain unclear, cantly increased on the symptomatic side during although the sympathetic nervous system is attacks.'* Marked increases in CIP amplitudes basi- thought to be the primary mediator of the impulses cally reflect an increase in pulsatile hlood volume. from the neck ro the ocular region. Ingestion of Fluorescein appearance time was studied to deter- alcohol may also trigger CPH, as is the case in clus- mine if there was a change in intraocular blood ter headache. flow. Almost identical fluorescein appearance time was seen in botb eyes, suggesting tbat the ampli- tude change is due to local changes in ocular circu- Absolute Effectjveness of Appropriate Doses of lation and not to a generalized vasodilation. This Indomethacin IS further supported by the lack of changes in internal carotid artety blood flow, blood pressure, The rapid treatment response to indomethacin and CSF pressure during an attack. forms part of the diagnostic criteria of CPH, allowing indomethacin to be used as a diagnostic test to differentiate CPH from other headaches. Intraocular Pressure The total response to indomethacjn'' is one of Increased intraocular pressure of 4 to 6 mm Hg the most impressive factors that identify CPH as a has also heen reported in CPH patients; this may unique entity. Tbe initial two patients studied by be due to the acute vasodilation that results from Sjaastad and Dale' reported acctylsalicyiic acid in the increased volume which occurs in the ocular doses of 6 to 8 g pet day as being tbe only medica- vascular bed during pain attacks.

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Pupitlometry that there is a blood-borne subsrance released from Patients with CPH tend to have a relatively small the ipsilateral carotid artery. The unilaterality of pupil on the symptomatic side, but pupiUometric CPH is also difficult ro explain by rhis proposed studies have not shown that a specific sympathetic mechanism, deficit is present. By contrast, patients suffering ("erebral angiography studies have failed to elu- from cluster headache often present wirh an asso- cidate any consistent finding that may produce ciated Horner's syndrome. tracrion on the blood vessels and so stimulare release of vasoacrive substances that can mediate pain. Nor was any evidence found of external Blood Tests compression of either the internal or common Many tests were performed, but all were found to carotid arteries, effectively disproving the hypothe- be within normal limits. No significant alterations sis that reduced cerebral blood flow subsequent to in any of the blood coagulation factors were found arterial compression is the cause of the headache, in parients with CPH. A conflicting hypothesis suggests that changes in cerebral blood flow patterns related to rhe opening of arreriovenous anastomoses may result in pain. Urinaty Histamine Excretion This hypothesis is based on rhe finding that reduc- Histamine has been closely associated with patho- tion in cerehral blood flow is known to occur with genesis oí cluster headache and therefore was sttid- administration of indomethacin." By exclusion, it ied in the CPH patients. No good correlation seems the mosr likely cause for precipitation of between the degree of histamine excretion and the attacks is via the aurononiic nervous system. The severity of the attacks could be observed. sympathetic system is most likely involved, as the parasympathetics to the end organs are hardly affected when the head is flexed, Sjaastad'' has concluded that in CPH it was most likely rhat pain and Proposed Mechanisms auronomic phenomena are triggered in parallel.

Since the establishment of this relatively new headache entity, many physiologic parameters have Case 1 been studied in CPH parienrs ro hecter understand and evaluate rhe mechanisms that couid be respon- A 53-year-oId wbite woman presented with rhe sible. Particularly the work of Ottar Sjaastad, these complaint of an intermittent sharp pain that was studies have led to speculation that CPH symptoms localized in the left maxillary and temporal are mediated either by autonomie impulses or vas- regions. The pain was described as occurring sev- cular factors. The latter could be associated with eral times a day and was often associated with either occlusion of arterial flow in the carotid or tearing in the ipsilareral eye and sweating. vertebral arteries, which may occur during head rotation or flexion, or vasoactive substances History released from cerebral (or neck) blood vessels. It has thus been theorized that mediation of The pain attacks were inirially felt as pain in rhe CPH symptoms is via the blood vessels and/ur upper premolar area, prompting the parienr ro visit autonomie impulses. Based on the response to the her dentist. The dentist proceeded ro perform roor anti-inflammatory medication indomcthacm, it has canal treatment on rhe premolar, which did not been suggested that the source of the pain in CPH substantially reduce the pain. The patient then is an active neurogenic inflammatory process consulted numerous physicians, including internal involving prostaglandins and vasoactive algesic medicine and neurologic specialists, and was even- substances, such as bradykinin and histamine. tually diagnosed as suffering from migraine. Trials However, this would not account for the changes using a number of migraine prophylactic medica- seen in the CIP amplitudes, the regular pattern of tions, including propranolol and amirripryline, CPH attacks, or the lack of response ro orher anti- were attempted without any benefit. The parient inflammatory medications. The fact chat CPH reported that her pain had been most successfully attacks can be precipitated with mechanical redticed by the use of 6 to 12 aspirin per day. The manipulation, without any refractory time pain could be triggered by rhe parient exrending berween the manipulation and the onser of the her head up and back, as would occur when the autonomie signs, does not support the hypothesis patient reached to get a teacup from a cupboard

Journal of Orofacial Pain 303 Deicanho

located above her head. The paiti had also been the only alleviating factor described by the patient noted ro be worse during cold weather. and was also reported to help stave off an attack. The patient had initially thought the pain was of dental origin and so had consulted a dentist, who Examination failed ro find any dental pathology, A physician The medical history revealed a family history of thought that trigeminal neuralgia was a possibility migraine, and the patient reported a previous his- and prescribed Dilantln (100 mg three times a tory of some intermittent migrainous symptoms. day), but this failed to produce any relief from the The review of systems was within normal hmits. attacks. The patient was referred to a neurosur- Psychosocial questioning revealed a happily mar- geon, who thought trigeminal neuralgia was an ried woman with two children and no major Stres- unlikely diagnosis, and was then referred on to an sors or financial problems. The Minnesota otolaryngologist. The ensuing examination, Multiphasic Personality Inventory ¡MMPI} was together with sinus radiography, proved negative administered and the scales were found Co be unel- for pathology, and it was suggested to the patient evated. The dental, stomatognathic, myofascial, that perhaps a temporomandibular joint (TMJ) cervical spine, and cranial nerve examinations problem was present. The patient then consulted a were all within normal limits. second dentist, who felt the problem was unlikely to be related to TMJ, and was referred to the Cedars-Sinai Anesthesia Pain Center. Impressions The intermittent chronic nature of the attacks, Examination occurring several times a day with associated autonomie features and triggerable by certain head The medical history and review of systems were all movements, led to a preliminary diagnosis of CPH. within normal limits. Dental, stomatognathic, The fact that only aspirin had been helpful was myofascial, cervical spine, and cranial nerve also .suggestive of CPH. The patient was started on screening examinations were negative or within a triai of 25 mg of indomethacin three times per normal limits. Psychosocial questioning was nega- day. The patient called 48 hours later to advise tive for possible depression and did not reveal a that she was pam free. The mitial controlhng dose need for psychometric evaluation. was found to be 125 mg per day, but she is now being maintained on 25 mg without any side effects. The patient has found that the longest she Impressions can stay off the indomethacin is 7 days before her Assessment of the chronic nature of the attacks, symptoms will return. combined with the unilaterality, regularity, duration, and lack of remission periods or triggers, led to a preliminary diagnosis of CPH. It was there- Case 2 fore thought that indomethacin trial may be helpful, and the patient was started on 75 mg A 67-year-old, retired white man presented with indomethacin SR per day, which was increased to the complaint of intermittent daily attacks of pain 75 mg twice per day after 4 days. One week later that was localized to the maxillary molar area. The the patient reported total relief from the attacks. pain was described as feeling as if a dentist were One month later he was still free of pain and was drilling the tooth without using local anesthesia. being maintained on 75 mg indomethacin SR per The attacks generally lasted 10 to 20 minutes, day. occurred regularly in the afternoon and then during the night, and occasionally woke him from sleep. Discussion History Chronic paroxysmal hemicrania is a rare head These pain attacks had been present ovet a 3-year pain syndrome, but one that should be considered period and no triggers had been identified that in the differential diagnosis of throbbing or boring would precipitate the attacks. There were no asso- pain in the maxillary region. This is particularly so ciated autonomie symptoms with the pain. When where there is a chronic daily pattern of multiple asked if anything aggravated the pain, the patient pain attacks of short duration. Dental pathology reported that stress could be a factor. Aspirin was should be ruled out Brst, and the use of local anes-

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Table 1 Organ System Classification of Headache Problems That Present as Toothache' Organ system Diagnosis Presence Quality In traerá nial Infarct Continuous Steady, du i i Tumor intermittent Steady, duii Extraerá nia i Sinusitis Conlinuoits Duil (vluscuioskeietai Myofaseiai pain Conlinuous Duil, aching Neurovascular Migraine intermittent Throbbing Ciuster intermittent Throbbmg Chronic paroxysmal hemicraria intermittent Throbbing Neuroganic Tngeminai neuralgia intermittent Sharp, shooting Pretrigeminal neuralgia Continuous Duii, aching Sympathetic Atypical odontaigia Continuous Duii, burning nervous system Psychogenic Psychogenic pain Vanabie Variable

thetic blocks may he required to ascertain that the in lower blood levels, and higher doses may he pain is not pulpal in origin. The presence of inter- required for equivalent effects to be achieved. If a mittent throbbing pains that are not detital in ori- positive effect occurs, the dosage can be varied up gin is generally suggestive of a neurovascular dis- (to a maximum 250 mg per day) or down to find a order (Table 1), Sinus, myofascial, and TMJ matntenance dose that keeps the pattent headache problems are also far more common than CPH free. The cyclic nature of CPH should be kept in and should be ruled out by the appropriate diag- mind, recognizing that the patient may enter a nostic procedures. period of exacerbation that requires dosage read- The chronic, unremitting nature of the attacks, justment. Tolerance does not seem to occur, which are often associated with autonomie signs indeed it has been reported that the amount of such as lacritnation, nasal stuffiness, conjunctival drug required may even decrease over time,-' injection, and rhinorrhea, should alert the clinician to the possibility of CPH. An indomethacin trial is the best test for establishing the diagnosis of CPH, Conclusion with a lack of response effectively ruling out CPH, Sjaastad'" has utilized indomethacin trial to rule Little is known about the nature of this uncom- out CPH whenever headache attack frequency mon facial pain condition, other than it is con- exceeds 4 occurrences in a 24-hour period. It trolled by indomethacin. At this stage the basis for should be noted that cerebrovascular disease and the absolute effect of this tnedication is unknown. other tntracranial pathology may cause headache Any proposed mechanism presented at this time to with simtlar features to CPH, and neuroimaging is explain CPH is also speculative. recommended wherever CPH-type symptoms are present.-*' The indomethacin effect, although absolute, is References dose dependent, and the dose should be titrated for each patient. For an indomethacin trial start 1, Bell WE, Toothaches of nonodontogenm origin, J Calif the patient with 75 mg per day and increase by 25 Dent Assocl976; 23:50-78, 2, Bdl WE, OrofacJal Pains, Classification, Diagnosis and mg every 3 days, up to a maximum of 250 mg. If Management, ed 4. Chicago: Year Book, 1989, no effect is noted within a few days, a CPH diag- 3, Graff-Radford SB, Headache problems that can present as nosis can be virtually excluded. The indomethacin toothache. Dental Gin North Am 1991;5(l):155-170, should be taken with food ro reduce the possibility 4, Sjaastad O, Dale I, A new (?) clinical headache entity of gastric side effects. For those parients in whom "Chronic Paroxysmal Hetnicrania," Acta Neurol Scand gastric side effects are significant, the use of either 1976;54:140-159, 5, Sjaastad O, Apfelbaum R, Casltey W, et al. Chronic the sustained release preparation or suppositories paroxysmal hemicrania (CPH), The clLnicd manifesta- may be helpful. However, these preparations result tions. A review, Upssala J Med Sci 1980;31(suppl):37-40.

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6, International Headache Society, Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain, Cephalalgia 19B8;7(suppl 8):1-96, Resumen 7, Antonaci F, Sjaastad O, Chronic paroxysmal hemicrania ¡CPH): A review of the clinical manifestations. Headache Hemicréneo Paroxistico Crónico Presentado como una 1989;29:648-656. Odontalgia 8, Ekbom K, Olivarius B, de Fine D, Chronic migrainous neuralgia—Diagnostic and therapeutic aspects. Headache El hemicréneo paroxistico crónico es un problema de cefalea intermitente caracterizado por paroxismos de dolor de 15 minu- 9, Russell D, Chronic paroxysmal hemicrania; severity, dura- tos de duración, aproximadamente. Los ataques están locsiiza- tion and time of occurrence of attacks. Cephalalgia dos UEuaimente en ia región fron tote m pora i y responden al uso 19S4;4:53-S6, de ia indometacina. Se presenta un grupo de síntomas que 10, Sjaastad 0, Russell D, Saunte C. Chronic paroxysmal definen ei hemicráneo paroxistico crónico, también se reportan hemicrania, VIII, The sweating pattern, Cephalalgia dos casos en ios cuaies se presentó la odontalgia como sín- 1983;3:45-S2, toma. Se enfatiza ei hecho de que ios clínicos deberian consid- 11, Sjaastadt O, Egge K, Horven I, et al. Chronic paroxysmal erar ei hemicráneo paroiíistico crónico en ei diagnóstico diferen- hemicrania: Mechanical precipitation of attacks. cial del doior orofacial. Headache 1979:19:31-36, 12, Sjaastad O, Russell D, Saunte C, Horven I. Chronic paroxysmal hemicrania, VI, Precipitation of attacks. Zusammenfassung Further studies on the precipitation mechanism, Cephalalgia I982;2:211-214, Zahnschmerzen als Symptom von chronischer paroxys- 13, Sjaastaii O, Saunte C, Graham JR, Chronic paroxysmal maler Hem i krame hemicrania. VII, Mechanical precipitation of attacks: New cases and localization of trigger points, Cephaialgia Chronische paroxysmale Hemikranie (CPH) ist ein schubweise verlaufender Typ von Kopfschmerzen, der durch ca. 1 5 Minuten 14, Sjaastad O, Aasly J, Fredriksen T, Wysocka-Bakowska andauernde Schmerzattacken im frontotemporalen Bereich MM. Chronic paroxysmal hemicrania, X, On the auto- charakterisiert. Typischerweise spricht die CPH auf nomie involvement, Cephalalgia 1986:6:113-124, indomethacin-Therapie an. Dieser Artikei unifasst eine 15, Price RW, Posner JB, Chronic paroxysmal hemicrania: A Lit eraturdu roh sieht über die Symptome der CPiH und berichtet disabling headache syndrome responding to indomethacin, über zwei Fälie, bei denen Zahnschmerzen im Bereich der AnnNeurol 197g;3(2}:183-184, Oberkieferprämoiaren und -molaren das Hauptsymptom waren 16, Jensen NB, Joensen P, Jensen J, Chronic paroxysmal hemi- Die Autoren weisen darajf hin, dass die CPH in die crania: Continued remission of symptoms after discontin- Differentia i diagnose orofacialer Schmerlen miteinbezogen wer- uation of indomethacin, Cephalalgia 1982;2:163-164, den muss. 17, Sjaastad O, Cluster Headache Syndrome, Major Problems in Neurology, vol 23, London: Saunders, 1992:291-352. 18, Horven I, Nornes H, Sjaastad O, Different corneal indentation pulse pattern in cluster headache and migraine. Neurology 1972;22i92-98, 19, Shigeno S, Fritschka E, Shigeno T, Brock M, Effects of Indomethacin on rCBF during and after focal cerebral ischemia in the cat. Stroke 1985;] 6:235-240, 20, Newman LC, Herskovitz S, Lipton RB, Solomon S, Chronic paroxysmal headache: Two cases with cere- hrovascular disease. Headache 1992;32:75-76. 21, Sjaastad O, Antonaci F. Chronic paroxysmal hemicrania: A case report. Longstanding remission in the chronic stage, Cephalalgia 1987^7:203-205.

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