An International Journal of Headache
doi:10.1111/j.1468-2982.2008.01576.x
CLINICAL CORRESPONDENCE
Orofacial cluster headache C Gaul1,2,3, AR Gantenbein1,4, UW Buettner4, DA Ettlin3 & PS Sándor1 1
Neurology Department, University Hospital Zurich, 3Clinic for Masticatory Disorders and Complete Dentures, Centre for Dental and Oral Medicine and Cranio-Maxillofacial Surgery, University of Zurich, Zurich, 4Neurology Department, Cantonal Hospital Aarau, Switzerland, and 2Department of Neurology, Martin-Luther-University Halle-Wittenberg, Halle, Germany
Charly Gaul, MD, Department of Neurology, Martin-Luther-University Halle-Wittenberg, Ernst-Grube-Straße 40, 06097 Halle, Germany. Tel. + 49 34 5557 2858, fax + 49 34 5557 2860, e-mail
[email protected] Received 27 July 2007, accepted 16 January 2008
Cluster headache is defined by the International Headache Society (IHS) as a severe or very severe unilateral pain in the orbital, supraorbital or temporal region with accompanying autonomic symptoms [International Classification of Headache Disorders (ICHD-II)] (1). The pain is usually present over 15–180 min and typically patients are pacing around during the headache phase. In patients with episodic cluster headache, there is typically a circadian rhythmicity with attacks predominantly at night and an additional circannual rhythmicity with clusters of attacks in spring and/or autumn. In chronic cases attack-free periods are lacking. We present two patients with episodic toothache, both of whom have been seen in a tertiary care headache clinic and by the dentist. Similar diagnostic problems are observed in migraine patients with predominantly pain localization in the second or third division of the trigeminal nerve, as we recently reported (2).
‘head-pressure’ and, in one attack, a radiating pain in the right ear. Conjunctival injection on the side of the pain was observed by one of the authors (A.R.G.) during an attack. The patient had first been seen by a dentist, who found no pathology. Neurological examination and routine blood tests performed during the pain-free interval were normal. Subcutaneous sumatriptan administered at the ED had little effect. However, high flow oxygen gave significant relief. Brain magnetic resonance imaging (MRI)/magnetic resonance angiography was unremarkable, including the cavernous sinus and pituitary gland. The patient was pain free on 100 mg prednisolone orally and remained pain free during tapering over 7–10 days. Thus, no prophylactic treatment was initiated. Follow-up after 1 year did not reveal any further similar pain episodes.
Case 2 Case 1 A 38-year-old, otherwise healthy man presented to the emergency department (ED) with an excruciating pain in the right mandible awaking him at 02.00 h. The attack lasted for approximately 1 h, during which he was agitated. He then went back to sleep. A similar kind of pain recurred again in the afternoon, with similar duration. This pattern continued for several days. In the beginning of these attacks, his pain was stabbing and of excruciating intensity. It was localized in the mandible and maxilla on the right side, around the molars. Other than this, he reported a slight accompanying
C.G. and A.R.G. have contributed equally.
© Blackwell Publishing Ltd Cephalalgia, 2008, 28, 903–905
A 68-year-old male with a history of 60 pack-years of smoking presented with nocturnal pain attacks restricted to the right mandible and described as toothache. A first nocturnal attack regularly occurred about 2 h after falling asleep. A second attack often followed several hours later. Attacks lasted for approximately 1 h and the pain was described as excruciating. Nasal congestion and conjunctival injection accompanied the dental pain. At presentation, he had been suffering from such episodes for several weeks. He had reported a bout of similar pain episodes 6 years earlier, with spontaneous remission after 6 weeks. Prior to his referral to the interdisciplinary orofacial pain consulting service, the right mandibular premolar was root canal treated and subsequently extracted, without benefit. Although 903
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pain maximum was always located in the right mandibular region, the diagnosis of probable ‘cluster headache type pain’ was made. Treatment with dihydroergotamine 1.25 mg at night was initiated, which resulted in pain relief after the second night. Neurological examination was unremarkable. Neuroimaging examination (MRI) was planned, but the patient cancelled the appointment, since his pain had responded to treatment. He was then lost to follow-up. We recently contacted his wife, who reported that the patient had remained symptom free until he passed away due to lung cancer 2 years after his consultation at our clinic. A post-mortem examination has not been performed. However, brain computed tomography scan just 1 day prior to the patient’s death was unremarkable and did not show brain or other metastases.
Discussion We describe two patients suffering from pain attacks with accompanying autonomic symptoms/signs who both—except for pain localization—fulfil the ICHD-II criteria of definite episodic cluster headache (ICHD-II 3.1.1) (1). Criterion B defines pain localization as ‘orbital, supraorbital and/or temporal’. Both of our patients, however, reported maximal pain clearly located in the mandible. Both patients were initially seen by a dentist, and, in one of them, several dental treatments and finally extraction were performed in an attempt to treat the pain. As pointed out by Bahra et al. in an analysis of 230 cluster headache patients, 45% had been seen by a dentist prior to diagnosis and 18% of all patients underwent tooth extractions, fillings, X-rays, maxillo-facial surgery, or received a splint brace (3). ‘Ectopic’ pain localization had previously been reported in cluster headache patients. Ekbom et al. (4) and later Campbell (5) have even differentiated an ‘upper syndrome’ and a ‘lower syndrome’ within the diagnostic entity of cluster headache, with maximal pain around and below the eye, respectively. Benoliel (6) has described cluster headache patients with orofacial localization of a ‘primary vascular-type craniofacial pain’, although evidence to confirm this hypothesized pathophysiological mechanism is not provided. Other factors accounting for the orofacial pain manifestation may be related to the somatotopic rearrangement of afferent nociceptive endings in the brainstem, where perioral regions are represented in the rostral part of the subnucleus caudalis and afferents of lateral face regions relay more caudally (commonly
referred to as an ‘onion-skin’ arrangement). This is evidenced clinically in that pain from the mandibular molars typically is referred to maxillary molars, which is not the case if pain localization is in the anterior mandible. Related to ‘orofacial cluster headache’, it is therefore not surprising that orbital pain may refer to maxillary or mandibular areas, particularly since the pain trigger is thought to be located in the central nervous system and is projected to peripheral receptive fields. Similarly, anatomical features such as the convergence of trigeminal and upper cervical pain roots in the cervical spinal root may likewise explain nuchal pain localization (7–10). The two patients reported here and similar cases in the literature (11) suggest that cluster headache may not be restricted to the first trigeminal division, but rather may also extend to the two orofacial (i.e. the maxillary and mandibular) divisions. In some patients, the pain seems to be exclusively restricted to the lower trigeminal roots without the usual cluster headache pain localization around the eye. Pain characteristics, time patterns, accompanying autonomous symptoms and the efficacy of established cluster headache therapy (oxygen and steroids in case 1 and dihydroergotamine in case 2) are all in favour of the diagnosis. Some authors have suggested a nitroglycerin provocative test in the diagnosis of migraine and cluster headache. In migraine, this test shows a sensitivity of around 80% (12). In cluster headache, the response to nitroglycerine provocation seems to depend on whether the patients are in or out of the bout (13). In all of 28 patients who were in the active period, a typical cluster attack was induced by nitroglycerin, but none of 15 of the same patients who were retested during remission responded with an attack then. Both patients responded well to therapy. Due to their atypical pain localization, these patients currently have to be diagnosed as ‘probable cluster headache’ (ICHD-II 3.4.1) according to ICHD-II, as they do not fulfil the localization criteria. Evidence put forward by Bahra et al. substantiates that almost every second cluster headache patient has been seen by a dentist and that one in five patients erroneously undergoes dental treatment (3). Since these patients present with typical symptoms of cluster headache, including periodicity, autonomic features as well as response to therapy, but merely with different pain localization, a classification as definite cluster headache might be more appropriate. This would require slightly extending pain localization in ICHD-II 3.4.1 criterion B (e.g. orofacial). © Blackwell Publishing Ltd Cephalalgia, 2008, 28, 903–905
Orofacial cluster headache Furthermore, there is a need to disseminate information about primary headache syndromes, especially cluster headaches, beyond neurologists in order to prevent inappropriate dental and medical treatment, but instead initiate appropriate pharmacological attack therapy with oxygen, ergotamine or triptans and prophylactic treatment with verapamil or lithium. We recently reported patients suffering from migraine in the second and third division of the trigeminal nerve (2). Together with the patients presented here suffering from a cluster headachelike syndrome with pain in the same localization, we suspect that a subset of orofacial pain syndromes can be diagnosed and should be treated as cluster headache and migraine. This might also contribute towards a better differentiation of ‘primary facial pain’. From a patient’s and a clinician’s point of view, it seems important to refine diagnostic criteria to avoid labels such as ‘atypical’ in clinical practice, in favour of a ‘proper diagnosis’. Finally, specific diagnoses enable better research.
Acknowledgements C.G. has been research fellow at the University of Zürich at the time of preparation of this work; he was supported by an MSD headache research grant.
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