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Orofacial pain due to trigeminal autonomic cephalgia with features of short-lasting neuralgiform headache attacks with conjunctival injection and tearing: a case report
Vol. 114 No. 4 October 2012
Orofacial pain due to trigeminal autonomic cephalgia with features of short-lasting neuralgiform headache attacks with conjunctival injection and tearing: a case report Ronald S. Brown, DDS, MS,a and Barry Pass, PhD, DDS,b Washington, DC HOWARD UNIVERSITY COLLEGE OF DENTISTRY AND SCHOOL OF ARTS AND SCIENCES AND GEORGETOWN UNIVERSITY MEDICAL CENTER
Background. We present a case of a 64-year-old woman with a presumptive diagnosis of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome with telangiectasia. Dental procedures were not successful in alleviating the condition. Results. The patient’s symptoms of short unilateral severe pain episodes abated after geographic relocation, although orofacial pain continued. Sphenoid sinus surgery further decreased the patient’s chronic pain complaints. The patient’s current pain condition is controlled with gabapentin therapy. Clinical Implications. Diagnostic, etiologic, and therapeutic issues related to SUNCT syndrome are discussed. This case represents the first case report of trigeminal autonomic cephalgia with SUNCT syndrome–like features illustrating possible problematic dental therapies. It is only the third SUNCT case report in the dental literature, and the third case reporting a correlation between SUNCT syndrome and sinusitis. (Oral Surg Oral Med Oral Pathol Oral Radiol 2012;114:e13-e19)
Patients with trigeminal autonomic cephalgias (TACs), such as short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), elicit pain symptoms of the orofacial complex; they may perceive this pain as tooth pain and therefore seek dental care. Inasmuch as many dentists treat chronic orofacial pain and dysfunction disorders of the head, neck, and facial pain, it is important for dentists to have an appreciation for TACs, such as SUNCT, and other similar diagnostic conditions, including short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), paroxysmal hemicrania (PH), and trigeminal neuralgia (TN) with lacrimation, to establish the correct diagnosis and avoid unnecessary, inappropriate, and invasive dental procedures.1– 4 SUNCT syndrome is a rare primary headache syndrome which was first described by Sjaastad et al. in 1978.5 Approximately 80 cases have been reported in the literature, although it is quite possible that this syndrome may be underdiagnosed and underreported. SUNCT tends to occur in slightly more men than a
Professor, Department of Oral Diagnostic Services, College of Dentistry, Howard University; Clinical Associate Professor, Department of Otolaryngology, Georgetown University Medical Center. b Professor, Department of Oral Diagnostic Services, Department of Physics, College of Dentistry, School of Arts and Sciences, Howard University. Received for publication Sep 28, 2011; returned for revision Feb 16, 2012; accepted for publication Feb 19, 2012. © 2012 Elsevier Inc. All rights reserved. 2212-4403/$ - see front matter doi:10.1016/j.oooo.2012.02.018
women and has an approximate age range of 35-65 years. A genetic familial predisposition has yet to be established. The pain in SUNCT is described as moderate to severe, unilateral pain episodes of short duration, localized to the orbital, supraorbital, or temporal sites or some combination of these sites, accompanied by one or more ipsilateral autonomic features (erythema, conjunctival injection, tearing).1–3,5–13 Balasubramaniam et al.1 reported that the pain episodes lasted 2-600 seconds, and Kruszewski et al.13 reported that the pain episodes were usually ⬍1 minute. These paroxysmal episodic pain attacks vary from less than once a day to more than 30-60 attacks per hour, with ipsilateral conjunctival injection with tearing (Headache Classification Committee of the International Headache Society, 2004).1,7,11,12 SUNCT attacks tend to occur during the day or evening, but rarely during the night. Unlike classic TN, there tends not to be a refractory period. The attacks typically are random and do not appear to follow any particular time pattern.1–3,5–13 SUNCT is associated with other TACs, such as cluster headache (CH) and PH,12 based on the classic clinical combination of head pain and activation of cranial autonomic efferents.1,12–14 TACs include SUNCT, SUNA, PH, and CH.1,11,15,16 At present, there is limited genetic association of these entities. The autonomic pain diagnostic entity, CH, is characterized by severe, mainly unilateral, pain attacks usually localized to the midface, orbital, and temporal regions and is accompanied by ipsilateral autonomic features. The pain may radiate to the midface, which has a number of oral and dental structures, and thus create a diagnostic dilemma as to the source of the pain. Autonomic fea-
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tures, such as rhinnorrhea, nasal congestion, eyelid edema, ptosis, miosis, facial sweating, and erythema, are infrequent though documented. The pain of CH is described as excruciating constant boring and burning. There is an approximate male-to-female ratio of 3:1, and the age of onset is in the twenties.1,12,13 A syndrome of short-lasting unilateral neuralgiform headache attacks with cranial autonomic features, SUNA, was also included within the second edition of the International Headache Classification.11,15 Cohen et al.11 reported a study reviewing a series of 43 SUNCT cases and 9 SUNA cases. All SUNCT patients had both conjunctival injection and tearing, whereas no SUNA patients had both of these autonomic symptoms. SUNCT and SUNA are listed within the larger diagnostic category of PH. The incidence of PH in the population is ⬃1 per 50,000. PH has many of the same characteristics as other TACs and is described as severe, short-lasting, strictly unilateral pain attacks which tend to be localized to the midface and is accompanied by 1 or more ipsilateral autonomic features. Owing to the location and symptoms, odontogenic etiology is part of the differential diagnosis. The severe pain of PH is described as boring or stabbing, and PH pain entities rarely occur at night nor interfere with sleep. Some patients with PH have reported experiencing pain between PH attacks. PH typically responds to indomethacin therapy, whereas SUNCT does not (which suggest differing pathophysiologies between PH and SUNCT).1,11,16 The pathophysiology of SUNCT has yet to be definitively defined. It is suggested that PH pain is mediated by the first division of the trigeminal nerve and supported by neurogenic inflammatory mechanisms involving intracranial and extracranial vasculature.1,12,17 A case of TAC with SUNCT-like features with noted telangiectasia is presented here. The patient’s shortlasting pain episodes resolved with time, though the patient still was experiencing chronic orofacial pain. Sinus surgery further decreased the patient’s chronic orofacial pain symptoms. This case is the third case report describing a possible association between SUNCT and sinus inflammation. To our knowledge, this is the third reported SUNCT-like case within the dental literature and the first documenting dental therapy for the treatment of pain symptoms related to a suspected diagnosis of SUNCT syndrome.2,18
CASE REPORT A 64-year-old woman reported to a private oral medicine clinician after a referral from her endodontist in May 2010. The patient noted a medical history positive for asthma, but was not taking any asthma medications at the time of the evaluation. She was previously prescribed steroids for her facial pain condition and secondarily had an infection of the
OOOO October 2012 stomach. The medical history was noted for spinal meningitis (1970), a hysterectomy and bladder lift procedure (1980), and sinus surgery (1997). The history was negative for exposure to cigarette smoking and alcohol use. Regarding family history, the patient reported that her mother had a history of TB and died of congestive heart failure at the age of 86 years, and that her father died of Parkinson disease at the age of 91 years. Her 2 brothers both had a history of heart disease, and a half-sister had a history of breast cancer. There was a family history of diabetes. Her drug history was notable for gabapentin (2,700 mg daily) and ibuprofen use for pain as well as a daily multivitamin supplement. The surgical history noted bilateral eyelid surgery in August 2006 and bone graft therapy of the maxillary right bicuspid area (September 2006), during the onset of the patient’s pain condition. Further surgeries included: endodontic therapy of the maxillary right cuspid (January 2007); endodontic therapy of the maxillary right lateral incisor (February 2007); apicoectomy surgery of the right maxillary cuspid (October 2007); a septoplasty, submucous resection, submucous resection of the inferior turbinate, and maxillary antrostomy (March 2007); gall bladder removal (2008); retinal detachment surgery of the right eye (April, 2009); foreign body removal in the maxillary right bicuspid area (July 2009); and extraction of the right maxillary cuspid (December 2009). A cone-beam computerized tomographic (CT) scan was read as negative in October 2009 and a CT scan in November 2009 ruled out a diagnosis of osteomylitis of the right maxilla. The next endodontist referred the patient to oral medicine in May of 2010 with a differential diagnosis that included complex regional pain syndrome (CRPS) and atypical facial pain (current terminology for this condition including atypical odontalgia, persistent orodental pain, persistent idiopathic facial pain, and persistent facial pain of unknown etiology). The pain began ⬃4 years before in September 2006, as the patient experienced extreme pain of the maxillary right area and noted that the right eye lid was swollen. She reported that her pain was episodic with 20-50 episodes daily. She reported some previous therapeutic success to local anesthesia spray procedures and that gabapentin therapy was unsuccessful. She described the pain as intense pain for 5 seconds with swelling of the right face, tearing of the right eye, and a runny nose. The tearing, runny nose, and swelling subside, but the right face and right eye remain reddened for another 5-10 minutes. She described the area just to the right of her nose as generally painful to touch. The patient experienced time periods between episodes which although not pain free, were noted to be considerably less painful than the intense pain during the short episodes. The episodes did not occur at night nor interfere with sleep. On examination, there was no lymphadenopathy. The patient experienced a pain episode during the examination. The pain was described as intense stabbing pain. Erythema, swelling, and telangiectasia of the right orbital and suborbital region were noted along with conjunctival injection with tearing (Figure 1). These autonomic findings diminished within 5-10 minutes. The clinical dental examination revealed that the right maxillary quadrant was edentulous with the exception of the right maxillary lateral and central
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Fig. 1. Photograph of the clinical appearance of the patient’s SUNCT episode. incisors. Recent periapical radiographs confirmed this finding and demonstrated a competent endodontic fill of the maxillary right lateral incisor and a small radiopaque area consistent with a submerged first molar root tip (Figure 2). The remaining oral tissues appeared to be within normal limits. A CT scan report of March 2010 reported right mild chronic sphenoid sinus mucosal thickening, and no evidence of a facial abscess, or osteomyelitis. Blood studies and chemistry of blood collected in November 2009 revealed only normal values. A neurology referral was suggested to the patient. However, the patient returned to her previous physician, who prescribed a 2-week regimen of intravenous penicillin, which improved her condition. In July of 2010, the patient relocated from the DC metropolitan area to a location within the mountains of Wyoming. The patient thought that the relocation improved her condition, because the severe orbital and suborbital pain episodes with autonomic signs ceased. The patient thought that the improvement was due to either a change in climate or the absence of exposure to lead paint. However, the patient still suffered from chronic orofacial pain, even with a daily regimen of 25 mg nortryptyline and 2,700 mg (3 times a day) gabapentin. A CT scan (November 2010) demonstrated circumferential mucosal thickening with mild thickening and sclerosis of the walls of the sphenoid sinus (Figure 3). In December 2010, an otolaryngologist performed an endoscopic right sphenoidotomy using extracranial stereotactic image guidance with endotracheal general anesthesia. A cyst-like–appearing area was discovered on the floor of the sphenoid which was curetted and submitted for histopathologic evaluation. The microscopic diagnosis was chronic sinusitis. The patient reported that her chronic orofacial pain condition was significantly improved after the surgical intervention. On follow-up in December 2011, 1 year
Fig. 2. A, Periapical radiograph of posterior maxilla. B, Periapical radiograph demonstrated a competent endodontic filling of the right maxillary lateral incisor. after surgery, the patient returned to the DC metropolitan area and reported that she is essentially asymptomatic maintained on 1,800 mg gabapentin.
DISCUSSION The differential diagnosis in this case included SUNCT, SUNA, PH, TN with lacrimation, and CRPS. According to Klasser and Balasubramaniam,3 the SUNCT diagnostic criteria include ⱖ20 attacks of unilateral orbital, supraorbital, or temporal stabbing or
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Fig. 3. A, Axial view of CT scan of right sphenoid sinus, demonstrating inflammation. B, Coronal view of CT scan of right sphenoid sinus, demonstrating inflammation. C, Sagittal view of CT scan of right sphenoid sinus, demonstrating inflammation.
pulsating pain lasting 5-240 seconds with accompanying ipsilateral conjunctival injection and lacrimation with a frequency of 3-200 attacks daily when other
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disorders have been ruled out.3 In that the present patient had both ipsilateral conjunctival injection and lacrimation, the diagnosis of SUNA appeared to be ruled out.1,11 The diagnostic criteria for PH include ⱖ20 attacks of severe unilateral orbital, supraorbital, or temporal pain lasting 2-30 minutes with headache accompanied by: 1) ipsilateral conjunctival injection and/or lacrimation; 2) ipsilateral nasal congestion and/or rhinorrhea; 3) eyelid edema; 4) ipsilateral forehead and facial sweating; and/or 5) ipsilateral miosis and/or ptosis. Furthermore, PH attacks are prevented completely by therapeutic doses of indomethacin, and PH symptomatology is not attributed to another disorder.4 Indomethacin therapy was never initiated in this case, and therefore a determination of this discriminating factor was left untried. The patient had been seeing a neurologist group before her referral to oral medicine, and those clinicians had not arrived at a reasonable diagnosis to account for the patient’s condition. The oral medicine clinician referred the patient to a specific neurologist with the suggestion that indomethacin and ganglionic block therapy be attempted to define the diagnosis. However, the patient relocated geographically, and therefore the recommended suggestions were not followed. The oral medicine clinician confirmed the endodontist’s diagnosis that the pain condition did not appear to be of odontogenic origin, and referred the patient for delineation of the neuropathic diagnosis and therapy. A summary of the patient’s condition from the case report data indicate that the attacks: 1) were short, ⬃5 seconds in duration; 2) were unilateral; 3) were noted for conjunctival injection and tearing; 4) resolved spontaneously; and 5) did not interfere with sleep. Based on these findings, the patient appears to have presented with TAC with SUNCT-like features, but without the indomethacin trial therapy, the diagnosis remains presumptive. In reading the literature, there appears to be some differences of opinion regarding the length of the pain episodes, as Balasubramaniam et al.1 reported that attacks may last up to 600 seconds (with a mean duration of 49 seconds). However, this discrepancy could be due to confusion regarding the secondary autonomic signs rather than the intense pain episodes. Also, the intense pain episodes of SUNCT may spontaneously remit, which occurred in the present case, whereas PH attacks are typically without remission periods beyond 1 month.3,4 Klasser and Balasubramaniam4 reported a case of TN with ipsilateral lacrimation. Their case was noted for a trigger point with short-duration paroxysmal pain, (12-15 seconds) and ipsilateral tearing, triggered pain which was abolished with local anesthesia, refractory periods, an excellent response to carbamazepine and baclofen, and no response to oxygen or indometh-
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acin. They noted the possibility that TN could develop into SUNCT, or that SUNCT and TN may be independent entities, and that TN may present with lacrimation. They noted 2 cases within the literature that were initially classic neurologic-type pain and developed over years into typical SUNCT. However, the present case did not demonstrate a trigger point, which would be necessary to define a diagnosis of TN.19 Saxen and Campbell20 reported a similar case with a diagnosis of CRPS. Their patient also experienced unilateral pain of the suborbital region with erythema and telangiectasia but without conjunctival injection or lacrimation. The present patient encountered several surgical procedures before the time of the onset of her symptoms, and previous trauma and surgical procedures are associated with CRPS20,21; however, there are significant difference between this case and CRPS, particularly the shortness of SUNCT attacks. This case was unique for at least 2 reasons. First, this case report of TAC with SUNCT-like features documents problematic dental therapy related to presumed failure of dental practitioners to differentiate nonodontogenic pain from odontogenic pain. Benoliel and Sharav2 reported a SUNCT case within the dental literature; however, the patient’s maxillary arch was edentulous, therefore ruling out an odontogenic etiology. De Siquera et al.18 reported a case with combined SUNCT and temporomandibular disorder (TMD)/myofascial pain. They reported successful treatment using balloon microcompression of the trigeminal ganglion in the treatment of SUNCT and injection and physical therapy in the treatment of TMD. Second, this was only the third case noting a possible association between SUNCT and sinusitis (although remission of SUNCT syndrome pain episodes occurred before the sinus surgery, which decreased the orofacial pain complaints). There is the possibility that the patient had two distinct conditions, sinusitis and TAC/SUNCT syndrome, which overlapped in time, or that the sinusitis condition precipitated the SUNCT-like features. Therefore, the patient’s geographic relocation to a drier climate could have positively influenced the patient’s sinusitis condition which may (or may not) have been important in the resolution of the SUNCT-like attacks. There is also the possibility that the patient had pain of odontogenic origin which required dental therapy during the same time that she was experiencing neuropathic orofacial pain and that the dental therapy was therefore necessary. There are a number of neuropathic, neuromuscular, and chronic pain disorders that have the ability to confuse dental and medical diagnosticians. Several of these chronic pain conditions have etiologies that are not fully comprehended, such as persistent idio-
ORIGINAL ARTICLE Brown and Pass e17
pathic facial pain, allodynia, SUNCT, SUNA, glossodynia, diabetic neuropathy, nutritional deficiency neuropathies, and CRPS.1,21 The failure of dentists to rule out pulpal (dental) etiology has the potential to result in inappropriate and destructive dental therapies.1,21 Nonetheless, it is possible that pain of dental origin could exist at the same time as other painful conditions. Furthermore, the major issue is that irreversible therapies (whether they are medical or dental) should not be initiated without the establishment of a diagnosis.21 The present case revealed that the patient underwent a number of what appear to be unnecessary dental therapies which appear to have resulted in unnecessary tooth loss. The patient saw a number of dental professionals before the referral by an endodontist to an oral medicine clinician. The failed periodontal graft procedures may have complicated the diagnostic process. The patient was also seen by several physicians, including several neurologists, who failed to arrive at a defined diagnosis during the time of the ongoing dental therapy. With the rarity of conditions such as PH, TN with lacrimation, SUNA, and SUNCT, the possibilities for incorrect diagnoses and therapies are increased. According to Goadsby and Lipton,12 in describing the etiopathology of SUNCT, both dura mater and large blood vessels are innervated largely by calcitonin gene–related peptide nerves that arise in the trigeminal ganglion. These bipolar nerves synapse in the trigeminal nucleus caudalis, including its caudal extension into the C1 and C2 dorsal horns of the cervical spinal cord. Pain signals are then transmitted via second-order neurons to the thalamus and then to the cortex for appreciation as pain. There is a functional connection to the premotor parasympathetic neurons of the seventh cranial (facial) nerve located in the superior salivatory nucleus (SSN). These neurons in turn give rise to preganglionic fibers that pass through the geniculate ganglion and synapse in the pterygopalatine and otic ganglia. Ganglionic transmission is by way of acetylcholine activating a nicotinic receptor. Postganglionic fibers transverse the greater superficial petrosal to innervate both of the cranial vessels and provide parasympathetic autonomic innervation to structures, such as the lacrimal glands and nasal mucosa, accounting for the marked parasympathetic autonomic features seen in these headaches. Kruszewski et al.13 reported increased heart rate and blood pressure during episodes of SUNCT. Such an increase in blood pressure could explain the occurrence of telangiectasia as a part of the symptomatology of the case presented.
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In the present case, the patient responded favorably to surgical therapy for sinus inflammation within the sphenoid sinus. Both Bichuetti et al.22 and Choi et al.23 reported resolution of SUNCT syndrome symptomatology after similar surgical intervention. Bichuetti et al.22 reported in a review of 21 patients that 5 had a history of sinusitis. However, Pareja and Sjaastad7 suggested, in a case series of SUNCT syndrome patients, that the presence of sinusitis is likely to be an incidental finding. It is possible that inflammation within anatomic structures such as the sinuses within proximity to particular sympathetic pathways (and such neural structures as the SSN, pterygopalatine and otic ganglia) may be involved in the etiology of SUNCT (and possibly other TACs). Furthermore, CT scans of the sinuses and associated structures may be helpful as a future investigative modality to determine a possible correlation between SUNCT and inflammatory conditions such as sinusitis. It is not determined whether or not inflammation of the sphenoid sinus contributed to the initiation or influence of the SUNCT syndrome condition, or that the patient merely had two distinct conditions: sinusitis and SUNCT syndrome. The presumed etiology of the patient’s present pain condition is continued sinusitis, and the patient is presently being treated by otolaryngologists and pain management specialists. Owing to the severe pain episodes in the past, the patient is reticent to taper her medications too quickly. Most drugs used in the treatment of other shortlasting headaches are not useful in SUNCT.1,24,25 Goadsby and Lipton12 noted that a great number of pharmacotherapeutics have failed to allieviate SUNCT syndrome, including aspirin, paracetamol, indomethacin, naproxen, ibuprofen, ergotamine, dihydroergotamine, sumatriptan, prednisone, methysergide, verapamil, valproate, lithium, propranolol, amitriptyline, and carbamazepine. However, success has been noted with such therapeutics as gabapentin, lamotrigine, and topiramate,26 –28 although other case reports indicate a lack of effectiveness with lamotrigine.1 In conclusion, a case with a presumed diagnosis of TAC with SUNCT-like features is presented. Owing to the difficulty of establishing the patient’s pain diagnosis, the patient thought that her facial pain was due to her dental condition, and she had a number of dental procedures which may have been unnecessary. A CT scan revealed thickening of the right sphenoid sinus consistent with sinusitis. After the remission of SUNCT pain episodes, but with remaining nonspecific orofacial pain, sinus surgery of the right sphenoid sinus decreased the patient’s pain. The patient’s pain has remained controlled with a decreased dosage of gabapentin for over the past year. The relationship between sinusitis and SUNCT remains unexplained. This case
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supports future investigation of inflammatory conditions of structures adjacent to orofacial sympathetic pathways as a possible etiologic factor of SUNCT syndrome. The authors thank Dr. Joseph Konzelman for his help with ascertaining the diagnosis and Dr. Richard Orlandi for providing the CT scans and for his surgical report. REFERENCES 1. Balasubramaniam R, Klasser GD, Delcanho R. Trigeminal autonomic cephalalgias. J Am Dent Assoc 2008;139:1616-24. 2. Benoliel R, Sharav Y. SUNCT syndrome: case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;85:158-61. 3. Klasser GD, Balasubramaniam R. Trigeminal autonomic cephalagias. Part 3: short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:763-71. 4. Klasser GD, Balasubramaniam R. Trigeminal autonomic cephalagias. Part 2: paroxysmal hemicrania. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:640-6. 5. Sjaastad O, Russell D, Horven I, Bunaes U. Multiple neuralgiform unilateral headache attacks associated with conjunctival injection and appearing in clusters: a nosological problem. In: Proceedings of the Annual Meeting, Scandinavian Migraine Society Aarhus 1978;31. 6. Pareja JA, Cuadrado ML. SUNCT syndrome: an update. Expert Opin Pharmacother 2005;6:591-9. 7. Pareja JA, Sjaastad O. SUNCT syndrome: a clinical review. Headache 1997;37:195-202. 8. Matharu MS, Cohen AS, Boes CJ, Goadsby PJ. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome: a review. Curr Pain Headache Rep 2003, a;7:308-18. 9. D’Andrea G, Granella F, Ghiotto N, Nappi G. Lamotrigine in the treatment of SUNCT syndrome. Neurology 2001;57:1723-5. 10. Wingerchuk DM, Nyquist PA, Rodriguez M, Dodick DW. Extratrigeminal short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT): new pathophysiologic entity or variation on a theme? Cephalalgia 2000;20:127-9. 11. Cohen AS, Matharu MS, Goadsby PJ. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA): a prospective clinical study of SUNCT and SUNA. Brain 2006;129:2746-60. 12. Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 1997;120:193-209. 13. Kruszewski P, Fasano ML, Brubakk AO, Shen JM, Sand T, Sjaastad O. Shortlasting, unilateral, neuralgiform headache attacks with conjunctival injection, tearing, and subclinical forehead sweating (“SUNCT” syndrome): II. Changes in heart rate and arterial blood pressure during pain paroxysms. Headache 1991;31:399-405. 14. May A, Goadsby PJ. The trigeminovascular system in humans: pathophysiological implications for primary headache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab 1999;19:115-27. 15. Matharu MS, Levy MJ, Merry RT, Goadsby PJ. SUNCT syndrome secondary to prolactinoma. J Neurol Neurosurg, Psychiatry 2003;74:1590-2. 16. Goadsby PJ, Cohen AS, Matharu MS. Trigeminal autonomic cephalagias: diagnosis and treatment. Curr Neurol Neurosci Rep 2007;7:117-25.
OOOO Volume 114, Number 4 17. Cohen AS. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Cephalgia 2007; 27:824-32. 18. de Siquera SR, Nóbrega JC, Teixeira MJ, de Siqueira JT. SUNCT syndrome associated with temporomandibular disorders: a case report. Cranio 2006;24:300-2. 19. Benoliel R, Sharav Y. Trigeminal neuralgia with lacrimation or SUNCT syndrome? Cephalalgia 1998;18:85-90. 20. Saxen MA, Campbell RL. An unusual case of sympathetically maintained facial pain complicated by telangiectasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:455-8. 21. Brown RS, Arm RN, Epstein JB, editors. Clinician’s guide for chronic orofacial pain. 2nd edit. Hamilton, Ontario: Decker; 2008. p. 1-80. 22. Bichuetti DB, Yamaoka WY, Bastos JRP, Carvalho Dde S. Bilateral SUNCT syndrome associated to chronic maxillary sinus disease. Arq Neuro Psiquiatr 2006;64:504-6. 23. Choi JY, Seo WK, Kim JH, Oh K, Yu SW. Symptomatic SUNCT syndrome associated with ipsilateral paranasal sinusitis. Headache 2008;48:1527-30.
ORIGINAL ARTICLE Brown and Pass e19 24. Pareja JA, Shen JM, Kruszewski P, Caballero V, Pamo M, Sjaastad O. SUNCT syndrome: duration, frequency, and temporal distribution of attacks. Headache 1996;36:161-5. 25. Ghose RR. SUNCT syndrome. Med J Aust 1995;162:667-8. 26. Cohen AS, Goadsby PJ. Proxysmal hemicrania responding to topiramate. J Neurol Neurosurg Psychiatry 2007;78: 96-7. 27. Kutschenko A, Liebetanz D. Meningioma causing gabapentinresponsive secondary SUNCT syndrome. J Headache Pain 2010; 11:359-61. 28. Graff-Radford SB. SUNCT syndrome responsive to gagapentin (Neurontin). Cephalalgia 2000;20:515-7.
Reprint requests: Dr. Ronald S. Brown HUCD, 600 W Street, NW, Room 406 Washington, DC 20059 [email protected]
Orofacial pain due to trigeminal autonomic cephalgia with features of short-lasting neuralgiform headache attacks with conjunctival injection and tearing: a case report Ronald S. Brown, DDS, MS,a and Barry Pass, PhD, DDS,b Washington, DC HOWARD UNIVERSITY COLLEGE OF DENTISTRY AND SCHOOL OF ARTS AND SCIENCES AND GEORGETOWN UNIVERSITY MEDICAL CENTER
Background. We present a case of a 64-year-old woman with a presumptive diagnosis of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome with telangiectasia. Dental procedures were not successful in alleviating the condition. Results. The patient’s symptoms of short unilateral severe pain episodes abated after geographic relocation, although orofacial pain continued. Sphenoid sinus surgery further decreased the patient’s chronic pain complaints. The patient’s current pain condition is controlled with gabapentin therapy. Clinical Implications. Diagnostic, etiologic, and therapeutic issues related to SUNCT syndrome are discussed. This case represents the first case report of trigeminal autonomic cephalgia with SUNCT syndrome–like features illustrating possible problematic dental therapies. It is only the third SUNCT case report in the dental literature, and the third case reporting a correlation between SUNCT syndrome and sinusitis. (Oral Surg Oral Med Oral Pathol Oral Radiol 2012;114:e13-e19)
Patients with trigeminal autonomic cephalgias (TACs), such as short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), elicit pain symptoms of the orofacial complex; they may perceive this pain as tooth pain and therefore seek dental care. Inasmuch as many dentists treat chronic orofacial pain and dysfunction disorders of the head, neck, and facial pain, it is important for dentists to have an appreciation for TACs, such as SUNCT, and other similar diagnostic conditions, including short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA), paroxysmal hemicrania (PH), and trigeminal neuralgia (TN) with lacrimation, to establish the correct diagnosis and avoid unnecessary, inappropriate, and invasive dental procedures.1– 4 SUNCT syndrome is a rare primary headache syndrome which was first described by Sjaastad et al. in 1978.5 Approximately 80 cases have been reported in the literature, although it is quite possible that this syndrome may be underdiagnosed and underreported. SUNCT tends to occur in slightly more men than a
Professor, Department of Oral Diagnostic Services, College of Dentistry, Howard University; Clinical Associate Professor, Department of Otolaryngology, Georgetown University Medical Center. b Professor, Department of Oral Diagnostic Services, Department of Physics, College of Dentistry, School of Arts and Sciences, Howard University. Received for publication Sep 28, 2011; returned for revision Feb 16, 2012; accepted for publication Feb 19, 2012. © 2012 Elsevier Inc. All rights reserved. 2212-4403/$ - see front matter doi:10.1016/j.oooo.2012.02.018
women and has an approximate age range of 35-65 years. A genetic familial predisposition has yet to be established. The pain in SUNCT is described as moderate to severe, unilateral pain episodes of short duration, localized to the orbital, supraorbital, or temporal sites or some combination of these sites, accompanied by one or more ipsilateral autonomic features (erythema, conjunctival injection, tearing).1–3,5–13 Balasubramaniam et al.1 reported that the pain episodes lasted 2-600 seconds, and Kruszewski et al.13 reported that the pain episodes were usually ⬍1 minute. These paroxysmal episodic pain attacks vary from less than once a day to more than 30-60 attacks per hour, with ipsilateral conjunctival injection with tearing (Headache Classification Committee of the International Headache Society, 2004).1,7,11,12 SUNCT attacks tend to occur during the day or evening, but rarely during the night. Unlike classic TN, there tends not to be a refractory period. The attacks typically are random and do not appear to follow any particular time pattern.1–3,5–13 SUNCT is associated with other TACs, such as cluster headache (CH) and PH,12 based on the classic clinical combination of head pain and activation of cranial autonomic efferents.1,12–14 TACs include SUNCT, SUNA, PH, and CH.1,11,15,16 At present, there is limited genetic association of these entities. The autonomic pain diagnostic entity, CH, is characterized by severe, mainly unilateral, pain attacks usually localized to the midface, orbital, and temporal regions and is accompanied by ipsilateral autonomic features. The pain may radiate to the midface, which has a number of oral and dental structures, and thus create a diagnostic dilemma as to the source of the pain. Autonomic fea-
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tures, such as rhinnorrhea, nasal congestion, eyelid edema, ptosis, miosis, facial sweating, and erythema, are infrequent though documented. The pain of CH is described as excruciating constant boring and burning. There is an approximate male-to-female ratio of 3:1, and the age of onset is in the twenties.1,12,13 A syndrome of short-lasting unilateral neuralgiform headache attacks with cranial autonomic features, SUNA, was also included within the second edition of the International Headache Classification.11,15 Cohen et al.11 reported a study reviewing a series of 43 SUNCT cases and 9 SUNA cases. All SUNCT patients had both conjunctival injection and tearing, whereas no SUNA patients had both of these autonomic symptoms. SUNCT and SUNA are listed within the larger diagnostic category of PH. The incidence of PH in the population is ⬃1 per 50,000. PH has many of the same characteristics as other TACs and is described as severe, short-lasting, strictly unilateral pain attacks which tend to be localized to the midface and is accompanied by 1 or more ipsilateral autonomic features. Owing to the location and symptoms, odontogenic etiology is part of the differential diagnosis. The severe pain of PH is described as boring or stabbing, and PH pain entities rarely occur at night nor interfere with sleep. Some patients with PH have reported experiencing pain between PH attacks. PH typically responds to indomethacin therapy, whereas SUNCT does not (which suggest differing pathophysiologies between PH and SUNCT).1,11,16 The pathophysiology of SUNCT has yet to be definitively defined. It is suggested that PH pain is mediated by the first division of the trigeminal nerve and supported by neurogenic inflammatory mechanisms involving intracranial and extracranial vasculature.1,12,17 A case of TAC with SUNCT-like features with noted telangiectasia is presented here. The patient’s shortlasting pain episodes resolved with time, though the patient still was experiencing chronic orofacial pain. Sinus surgery further decreased the patient’s chronic orofacial pain symptoms. This case is the third case report describing a possible association between SUNCT and sinus inflammation. To our knowledge, this is the third reported SUNCT-like case within the dental literature and the first documenting dental therapy for the treatment of pain symptoms related to a suspected diagnosis of SUNCT syndrome.2,18
CASE REPORT A 64-year-old woman reported to a private oral medicine clinician after a referral from her endodontist in May 2010. The patient noted a medical history positive for asthma, but was not taking any asthma medications at the time of the evaluation. She was previously prescribed steroids for her facial pain condition and secondarily had an infection of the
OOOO October 2012 stomach. The medical history was noted for spinal meningitis (1970), a hysterectomy and bladder lift procedure (1980), and sinus surgery (1997). The history was negative for exposure to cigarette smoking and alcohol use. Regarding family history, the patient reported that her mother had a history of TB and died of congestive heart failure at the age of 86 years, and that her father died of Parkinson disease at the age of 91 years. Her 2 brothers both had a history of heart disease, and a half-sister had a history of breast cancer. There was a family history of diabetes. Her drug history was notable for gabapentin (2,700 mg daily) and ibuprofen use for pain as well as a daily multivitamin supplement. The surgical history noted bilateral eyelid surgery in August 2006 and bone graft therapy of the maxillary right bicuspid area (September 2006), during the onset of the patient’s pain condition. Further surgeries included: endodontic therapy of the maxillary right cuspid (January 2007); endodontic therapy of the maxillary right lateral incisor (February 2007); apicoectomy surgery of the right maxillary cuspid (October 2007); a septoplasty, submucous resection, submucous resection of the inferior turbinate, and maxillary antrostomy (March 2007); gall bladder removal (2008); retinal detachment surgery of the right eye (April, 2009); foreign body removal in the maxillary right bicuspid area (July 2009); and extraction of the right maxillary cuspid (December 2009). A cone-beam computerized tomographic (CT) scan was read as negative in October 2009 and a CT scan in November 2009 ruled out a diagnosis of osteomylitis of the right maxilla. The next endodontist referred the patient to oral medicine in May of 2010 with a differential diagnosis that included complex regional pain syndrome (CRPS) and atypical facial pain (current terminology for this condition including atypical odontalgia, persistent orodental pain, persistent idiopathic facial pain, and persistent facial pain of unknown etiology). The pain began ⬃4 years before in September 2006, as the patient experienced extreme pain of the maxillary right area and noted that the right eye lid was swollen. She reported that her pain was episodic with 20-50 episodes daily. She reported some previous therapeutic success to local anesthesia spray procedures and that gabapentin therapy was unsuccessful. She described the pain as intense pain for 5 seconds with swelling of the right face, tearing of the right eye, and a runny nose. The tearing, runny nose, and swelling subside, but the right face and right eye remain reddened for another 5-10 minutes. She described the area just to the right of her nose as generally painful to touch. The patient experienced time periods between episodes which although not pain free, were noted to be considerably less painful than the intense pain during the short episodes. The episodes did not occur at night nor interfere with sleep. On examination, there was no lymphadenopathy. The patient experienced a pain episode during the examination. The pain was described as intense stabbing pain. Erythema, swelling, and telangiectasia of the right orbital and suborbital region were noted along with conjunctival injection with tearing (Figure 1). These autonomic findings diminished within 5-10 minutes. The clinical dental examination revealed that the right maxillary quadrant was edentulous with the exception of the right maxillary lateral and central
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Fig. 1. Photograph of the clinical appearance of the patient’s SUNCT episode. incisors. Recent periapical radiographs confirmed this finding and demonstrated a competent endodontic fill of the maxillary right lateral incisor and a small radiopaque area consistent with a submerged first molar root tip (Figure 2). The remaining oral tissues appeared to be within normal limits. A CT scan report of March 2010 reported right mild chronic sphenoid sinus mucosal thickening, and no evidence of a facial abscess, or osteomyelitis. Blood studies and chemistry of blood collected in November 2009 revealed only normal values. A neurology referral was suggested to the patient. However, the patient returned to her previous physician, who prescribed a 2-week regimen of intravenous penicillin, which improved her condition. In July of 2010, the patient relocated from the DC metropolitan area to a location within the mountains of Wyoming. The patient thought that the relocation improved her condition, because the severe orbital and suborbital pain episodes with autonomic signs ceased. The patient thought that the improvement was due to either a change in climate or the absence of exposure to lead paint. However, the patient still suffered from chronic orofacial pain, even with a daily regimen of 25 mg nortryptyline and 2,700 mg (3 times a day) gabapentin. A CT scan (November 2010) demonstrated circumferential mucosal thickening with mild thickening and sclerosis of the walls of the sphenoid sinus (Figure 3). In December 2010, an otolaryngologist performed an endoscopic right sphenoidotomy using extracranial stereotactic image guidance with endotracheal general anesthesia. A cyst-like–appearing area was discovered on the floor of the sphenoid which was curetted and submitted for histopathologic evaluation. The microscopic diagnosis was chronic sinusitis. The patient reported that her chronic orofacial pain condition was significantly improved after the surgical intervention. On follow-up in December 2011, 1 year
Fig. 2. A, Periapical radiograph of posterior maxilla. B, Periapical radiograph demonstrated a competent endodontic filling of the right maxillary lateral incisor. after surgery, the patient returned to the DC metropolitan area and reported that she is essentially asymptomatic maintained on 1,800 mg gabapentin.
DISCUSSION The differential diagnosis in this case included SUNCT, SUNA, PH, TN with lacrimation, and CRPS. According to Klasser and Balasubramaniam,3 the SUNCT diagnostic criteria include ⱖ20 attacks of unilateral orbital, supraorbital, or temporal stabbing or
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Fig. 3. A, Axial view of CT scan of right sphenoid sinus, demonstrating inflammation. B, Coronal view of CT scan of right sphenoid sinus, demonstrating inflammation. C, Sagittal view of CT scan of right sphenoid sinus, demonstrating inflammation.
pulsating pain lasting 5-240 seconds with accompanying ipsilateral conjunctival injection and lacrimation with a frequency of 3-200 attacks daily when other
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disorders have been ruled out.3 In that the present patient had both ipsilateral conjunctival injection and lacrimation, the diagnosis of SUNA appeared to be ruled out.1,11 The diagnostic criteria for PH include ⱖ20 attacks of severe unilateral orbital, supraorbital, or temporal pain lasting 2-30 minutes with headache accompanied by: 1) ipsilateral conjunctival injection and/or lacrimation; 2) ipsilateral nasal congestion and/or rhinorrhea; 3) eyelid edema; 4) ipsilateral forehead and facial sweating; and/or 5) ipsilateral miosis and/or ptosis. Furthermore, PH attacks are prevented completely by therapeutic doses of indomethacin, and PH symptomatology is not attributed to another disorder.4 Indomethacin therapy was never initiated in this case, and therefore a determination of this discriminating factor was left untried. The patient had been seeing a neurologist group before her referral to oral medicine, and those clinicians had not arrived at a reasonable diagnosis to account for the patient’s condition. The oral medicine clinician referred the patient to a specific neurologist with the suggestion that indomethacin and ganglionic block therapy be attempted to define the diagnosis. However, the patient relocated geographically, and therefore the recommended suggestions were not followed. The oral medicine clinician confirmed the endodontist’s diagnosis that the pain condition did not appear to be of odontogenic origin, and referred the patient for delineation of the neuropathic diagnosis and therapy. A summary of the patient’s condition from the case report data indicate that the attacks: 1) were short, ⬃5 seconds in duration; 2) were unilateral; 3) were noted for conjunctival injection and tearing; 4) resolved spontaneously; and 5) did not interfere with sleep. Based on these findings, the patient appears to have presented with TAC with SUNCT-like features, but without the indomethacin trial therapy, the diagnosis remains presumptive. In reading the literature, there appears to be some differences of opinion regarding the length of the pain episodes, as Balasubramaniam et al.1 reported that attacks may last up to 600 seconds (with a mean duration of 49 seconds). However, this discrepancy could be due to confusion regarding the secondary autonomic signs rather than the intense pain episodes. Also, the intense pain episodes of SUNCT may spontaneously remit, which occurred in the present case, whereas PH attacks are typically without remission periods beyond 1 month.3,4 Klasser and Balasubramaniam4 reported a case of TN with ipsilateral lacrimation. Their case was noted for a trigger point with short-duration paroxysmal pain, (12-15 seconds) and ipsilateral tearing, triggered pain which was abolished with local anesthesia, refractory periods, an excellent response to carbamazepine and baclofen, and no response to oxygen or indometh-
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acin. They noted the possibility that TN could develop into SUNCT, or that SUNCT and TN may be independent entities, and that TN may present with lacrimation. They noted 2 cases within the literature that were initially classic neurologic-type pain and developed over years into typical SUNCT. However, the present case did not demonstrate a trigger point, which would be necessary to define a diagnosis of TN.19 Saxen and Campbell20 reported a similar case with a diagnosis of CRPS. Their patient also experienced unilateral pain of the suborbital region with erythema and telangiectasia but without conjunctival injection or lacrimation. The present patient encountered several surgical procedures before the time of the onset of her symptoms, and previous trauma and surgical procedures are associated with CRPS20,21; however, there are significant difference between this case and CRPS, particularly the shortness of SUNCT attacks. This case was unique for at least 2 reasons. First, this case report of TAC with SUNCT-like features documents problematic dental therapy related to presumed failure of dental practitioners to differentiate nonodontogenic pain from odontogenic pain. Benoliel and Sharav2 reported a SUNCT case within the dental literature; however, the patient’s maxillary arch was edentulous, therefore ruling out an odontogenic etiology. De Siquera et al.18 reported a case with combined SUNCT and temporomandibular disorder (TMD)/myofascial pain. They reported successful treatment using balloon microcompression of the trigeminal ganglion in the treatment of SUNCT and injection and physical therapy in the treatment of TMD. Second, this was only the third case noting a possible association between SUNCT and sinusitis (although remission of SUNCT syndrome pain episodes occurred before the sinus surgery, which decreased the orofacial pain complaints). There is the possibility that the patient had two distinct conditions, sinusitis and TAC/SUNCT syndrome, which overlapped in time, or that the sinusitis condition precipitated the SUNCT-like features. Therefore, the patient’s geographic relocation to a drier climate could have positively influenced the patient’s sinusitis condition which may (or may not) have been important in the resolution of the SUNCT-like attacks. There is also the possibility that the patient had pain of odontogenic origin which required dental therapy during the same time that she was experiencing neuropathic orofacial pain and that the dental therapy was therefore necessary. There are a number of neuropathic, neuromuscular, and chronic pain disorders that have the ability to confuse dental and medical diagnosticians. Several of these chronic pain conditions have etiologies that are not fully comprehended, such as persistent idio-
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pathic facial pain, allodynia, SUNCT, SUNA, glossodynia, diabetic neuropathy, nutritional deficiency neuropathies, and CRPS.1,21 The failure of dentists to rule out pulpal (dental) etiology has the potential to result in inappropriate and destructive dental therapies.1,21 Nonetheless, it is possible that pain of dental origin could exist at the same time as other painful conditions. Furthermore, the major issue is that irreversible therapies (whether they are medical or dental) should not be initiated without the establishment of a diagnosis.21 The present case revealed that the patient underwent a number of what appear to be unnecessary dental therapies which appear to have resulted in unnecessary tooth loss. The patient saw a number of dental professionals before the referral by an endodontist to an oral medicine clinician. The failed periodontal graft procedures may have complicated the diagnostic process. The patient was also seen by several physicians, including several neurologists, who failed to arrive at a defined diagnosis during the time of the ongoing dental therapy. With the rarity of conditions such as PH, TN with lacrimation, SUNA, and SUNCT, the possibilities for incorrect diagnoses and therapies are increased. According to Goadsby and Lipton,12 in describing the etiopathology of SUNCT, both dura mater and large blood vessels are innervated largely by calcitonin gene–related peptide nerves that arise in the trigeminal ganglion. These bipolar nerves synapse in the trigeminal nucleus caudalis, including its caudal extension into the C1 and C2 dorsal horns of the cervical spinal cord. Pain signals are then transmitted via second-order neurons to the thalamus and then to the cortex for appreciation as pain. There is a functional connection to the premotor parasympathetic neurons of the seventh cranial (facial) nerve located in the superior salivatory nucleus (SSN). These neurons in turn give rise to preganglionic fibers that pass through the geniculate ganglion and synapse in the pterygopalatine and otic ganglia. Ganglionic transmission is by way of acetylcholine activating a nicotinic receptor. Postganglionic fibers transverse the greater superficial petrosal to innervate both of the cranial vessels and provide parasympathetic autonomic innervation to structures, such as the lacrimal glands and nasal mucosa, accounting for the marked parasympathetic autonomic features seen in these headaches. Kruszewski et al.13 reported increased heart rate and blood pressure during episodes of SUNCT. Such an increase in blood pressure could explain the occurrence of telangiectasia as a part of the symptomatology of the case presented.
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In the present case, the patient responded favorably to surgical therapy for sinus inflammation within the sphenoid sinus. Both Bichuetti et al.22 and Choi et al.23 reported resolution of SUNCT syndrome symptomatology after similar surgical intervention. Bichuetti et al.22 reported in a review of 21 patients that 5 had a history of sinusitis. However, Pareja and Sjaastad7 suggested, in a case series of SUNCT syndrome patients, that the presence of sinusitis is likely to be an incidental finding. It is possible that inflammation within anatomic structures such as the sinuses within proximity to particular sympathetic pathways (and such neural structures as the SSN, pterygopalatine and otic ganglia) may be involved in the etiology of SUNCT (and possibly other TACs). Furthermore, CT scans of the sinuses and associated structures may be helpful as a future investigative modality to determine a possible correlation between SUNCT and inflammatory conditions such as sinusitis. It is not determined whether or not inflammation of the sphenoid sinus contributed to the initiation or influence of the SUNCT syndrome condition, or that the patient merely had two distinct conditions: sinusitis and SUNCT syndrome. The presumed etiology of the patient’s present pain condition is continued sinusitis, and the patient is presently being treated by otolaryngologists and pain management specialists. Owing to the severe pain episodes in the past, the patient is reticent to taper her medications too quickly. Most drugs used in the treatment of other shortlasting headaches are not useful in SUNCT.1,24,25 Goadsby and Lipton12 noted that a great number of pharmacotherapeutics have failed to allieviate SUNCT syndrome, including aspirin, paracetamol, indomethacin, naproxen, ibuprofen, ergotamine, dihydroergotamine, sumatriptan, prednisone, methysergide, verapamil, valproate, lithium, propranolol, amitriptyline, and carbamazepine. However, success has been noted with such therapeutics as gabapentin, lamotrigine, and topiramate,26 –28 although other case reports indicate a lack of effectiveness with lamotrigine.1 In conclusion, a case with a presumed diagnosis of TAC with SUNCT-like features is presented. Owing to the difficulty of establishing the patient’s pain diagnosis, the patient thought that her facial pain was due to her dental condition, and she had a number of dental procedures which may have been unnecessary. A CT scan revealed thickening of the right sphenoid sinus consistent with sinusitis. After the remission of SUNCT pain episodes, but with remaining nonspecific orofacial pain, sinus surgery of the right sphenoid sinus decreased the patient’s pain. The patient’s pain has remained controlled with a decreased dosage of gabapentin for over the past year. The relationship between sinusitis and SUNCT remains unexplained. This case
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supports future investigation of inflammatory conditions of structures adjacent to orofacial sympathetic pathways as a possible etiologic factor of SUNCT syndrome. The authors thank Dr. Joseph Konzelman for his help with ascertaining the diagnosis and Dr. Richard Orlandi for providing the CT scans and for his surgical report. REFERENCES 1. Balasubramaniam R, Klasser GD, Delcanho R. Trigeminal autonomic cephalalgias. J Am Dent Assoc 2008;139:1616-24. 2. Benoliel R, Sharav Y. SUNCT syndrome: case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;85:158-61. 3. Klasser GD, Balasubramaniam R. Trigeminal autonomic cephalagias. Part 3: short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:763-71. 4. Klasser GD, Balasubramaniam R. Trigeminal autonomic cephalagias. Part 2: paroxysmal hemicrania. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2007;104:640-6. 5. Sjaastad O, Russell D, Horven I, Bunaes U. Multiple neuralgiform unilateral headache attacks associated with conjunctival injection and appearing in clusters: a nosological problem. In: Proceedings of the Annual Meeting, Scandinavian Migraine Society Aarhus 1978;31. 6. Pareja JA, Cuadrado ML. SUNCT syndrome: an update. Expert Opin Pharmacother 2005;6:591-9. 7. Pareja JA, Sjaastad O. SUNCT syndrome: a clinical review. Headache 1997;37:195-202. 8. Matharu MS, Cohen AS, Boes CJ, Goadsby PJ. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome: a review. Curr Pain Headache Rep 2003, a;7:308-18. 9. D’Andrea G, Granella F, Ghiotto N, Nappi G. Lamotrigine in the treatment of SUNCT syndrome. Neurology 2001;57:1723-5. 10. Wingerchuk DM, Nyquist PA, Rodriguez M, Dodick DW. Extratrigeminal short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT): new pathophysiologic entity or variation on a theme? Cephalalgia 2000;20:127-9. 11. Cohen AS, Matharu MS, Goadsby PJ. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) or cranial autonomic features (SUNA): a prospective clinical study of SUNCT and SUNA. Brain 2006;129:2746-60. 12. Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain 1997;120:193-209. 13. Kruszewski P, Fasano ML, Brubakk AO, Shen JM, Sand T, Sjaastad O. Shortlasting, unilateral, neuralgiform headache attacks with conjunctival injection, tearing, and subclinical forehead sweating (“SUNCT” syndrome): II. Changes in heart rate and arterial blood pressure during pain paroxysms. Headache 1991;31:399-405. 14. May A, Goadsby PJ. The trigeminovascular system in humans: pathophysiological implications for primary headache syndromes of the neural influences on the cerebral circulation. J Cereb Blood Flow Metab 1999;19:115-27. 15. Matharu MS, Levy MJ, Merry RT, Goadsby PJ. SUNCT syndrome secondary to prolactinoma. J Neurol Neurosurg, Psychiatry 2003;74:1590-2. 16. Goadsby PJ, Cohen AS, Matharu MS. Trigeminal autonomic cephalagias: diagnosis and treatment. Curr Neurol Neurosci Rep 2007;7:117-25.
OOOO Volume 114, Number 4 17. Cohen AS. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. Cephalgia 2007; 27:824-32. 18. de Siquera SR, Nóbrega JC, Teixeira MJ, de Siqueira JT. SUNCT syndrome associated with temporomandibular disorders: a case report. Cranio 2006;24:300-2. 19. Benoliel R, Sharav Y. Trigeminal neuralgia with lacrimation or SUNCT syndrome? Cephalalgia 1998;18:85-90. 20. Saxen MA, Campbell RL. An unusual case of sympathetically maintained facial pain complicated by telangiectasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:455-8. 21. Brown RS, Arm RN, Epstein JB, editors. Clinician’s guide for chronic orofacial pain. 2nd edit. Hamilton, Ontario: Decker; 2008. p. 1-80. 22. Bichuetti DB, Yamaoka WY, Bastos JRP, Carvalho Dde S. Bilateral SUNCT syndrome associated to chronic maxillary sinus disease. Arq Neuro Psiquiatr 2006;64:504-6. 23. Choi JY, Seo WK, Kim JH, Oh K, Yu SW. Symptomatic SUNCT syndrome associated with ipsilateral paranasal sinusitis. Headache 2008;48:1527-30.
ORIGINAL ARTICLE Brown and Pass e19 24. Pareja JA, Shen JM, Kruszewski P, Caballero V, Pamo M, Sjaastad O. SUNCT syndrome: duration, frequency, and temporal distribution of attacks. Headache 1996;36:161-5. 25. Ghose RR. SUNCT syndrome. Med J Aust 1995;162:667-8. 26. Cohen AS, Goadsby PJ. Proxysmal hemicrania responding to topiramate. J Neurol Neurosurg Psychiatry 2007;78: 96-7. 27. Kutschenko A, Liebetanz D. Meningioma causing gabapentinresponsive secondary SUNCT syndrome. J Headache Pain 2010; 11:359-61. 28. Graff-Radford SB. SUNCT syndrome responsive to gagapentin (Neurontin). Cephalalgia 2000;20:515-7.
Reprint requests: Dr. Ronald S. Brown HUCD, 600 W Street, NW, Room 406 Washington, DC 20059 [email protected]