mise à jour du
14 février 2005
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome treated with microvascular decompression of the trigeminal nerve: case report
Alfonso Lagares, Pedro A. Gomez, Angel Pérez-Nuñez,
Ramiro D. Lobato, Ana Ramos
  Department of Neurosurgery, Hospital 12 de Octubre, Madrid, Spain  


Secondary yawning pain in patients with cranial neuralgia
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a very rare disorder characterized by short-lasting neuralgiform unilateral pain affecting the orbital-periorbital area, lasting for 5 to 250 seconds, sometimes precipitated by stimuli in the trigeminal area, and associated with autonomic phenomena consisting mainly of conjunctival injection, tearing, and rhinorrhea (19). Although many different pharmacological treatments have been tried, no single treatment has achieved a sure and definite response in these patients. There also have been reports of patients treated with surgery, with variable results. To our knowledge, microvascular decompression has been reported as successful in only two previous cases. However, the case of a patient who did not respond to two different attempts of trigeminal root decompression also has been reported. We present the case of a patient affected by this syndrome who responded to microvascular decompression.
    A 56-year-old woman was referred to our clinic because she had experienced pain in the distribution of the first left trigeminal branch during the previous 2 years. The pain was described as electric or lancinating, spreading over the left orbit and forehead as well as the left frontal and temporal areas of the hair. She experienced paroxysms lasting from a few seconds to 1 to 2 minutes superimposed over a dull sensation of pain involving the same territory. The paroxysms were triggered by touching the eye or the left side of the face, chewing, yawning, washing her hair, and even light. Although the paroxysms were triggered by a light touch or chewing, she was able to talk or touch herself while having the paroxysm. Sometimes, she also had pain in her throat or left ear. During attacks of pain, she experienced tearing and ipsilateral conjunctival injection, eyelid edema and rhinorrhea, as well as intense photophobia. The attacks had no refractory period and normally disappeared while the patient was sleeping.
    She had been administered different treatments, with no response, before coming to our clinic. Carbamazepine, gabapentin, topiramate, and phenytoin had been tried with no response at all. We attempted indomethacin, oxygen, and another trial of carbamazepine and baclofen also without response. The patient was hypertense and could not tolerate verapamil because it increased or even provoked pain paroxysms.
    Neurological examination results were normal, with no trigeminal hypesthesia and a normal corneal reflex. A magnetic resonance imaging (MRI) examination revealed a vascular structure compressing and distorting the left trigeminal root. Because no medical treatment had been useful and the patient was seriously incapacitated by photophobia and pain, we decided to perform a microvascular decompression of the trigeminal root.
At surgery, there was clear compression of the trigeminal root by a superior cerebellar artery loop that was resolved by interposing a Teflon patch. The patient awoke from the operation without pain, and all the accompanying signs and symptoms, such as photophobia, had disappeared. The postoperative course was uneventful, and a control MRI scan showed resolution of the trigeminal root compression. Two years after treatment, the patient remains asymptomatic.
    Autonomic features, rare in neuropathic pain, are more common in vascular pain syndromes. However, the association between a neuritic or neuralgiform pain and autonomic features such as tearing or conjunctival injection are present in several head pain syndromes, such as cluster headache, paroxysmal hemicrania, hemicrania continua, and SUNCT syndrome, which are grouped as the so-called trigeminal autonomic cephalalgias. These short-lasting headaches are characterized by unilateral trigeminal pain occurring in association with prominent ipsilateral cranial autonomic features.
  Different pathophysiological mechanisms have been proposed to explain the connection between pain in the trigeminal territory and autonomic features. Goadsby and Lipton (6) hypothesized that these headaches are characterized by an activation between trigeminal afferents, which would give rise to pain, and cranial parasympathetic efferents, which would be responsible for autonomic features, via the trigeminal-autonomic activation reflex. The pathway of this reflex could consist of a brainstem connection between the trigeminal and the parasympathetic outflow accompanying the VIIth cranial nerve. There is experimental evidence supporting the connection between trigeminal afferents and changes in parasympathetic outflow, such as occurrence of increased cerebral and extracerebral blood flow via vasodilation after trigeminal ganglion stimulation in animals and humans. Moreover, these cranial autonomic symptoms could be prominent in trigeminal autonomic cephalalgias resulting from a possible central disinhibition of the trigeminal-autonomic reflex. Positron emission tomography and functional MRI studies performed during pain bouts have evidenced abnormal ipsilateral hypothalamic activation in patients with cluster headache and SUNCT syndrome (14, 15). These findings all seem to suggest that there are hypothalamic-trigeminal connections and that the hypothalamus has a modulatory role on the nociceptive and autonomic pathways. Thus, a dysfunction involving these structures could be responsible for the association of facial pain and autonomic features characteristic of these headaches.
 SUNCT syndrome, first described by Sjaastad et al. (19) in 1989, is characterized by unilateral facial pain, maximal in the ophthalmic distribution of the trigeminal nerve, especially in the orbital or periorbital regions, forehead, and temple (6, 10, 13, 17, 19). The individual attacks are brief, lasting from 5 to 250 seconds, although attacks lasting hours have been described. Patients can precipitate attacks by touching trigger zones within the trigeminal territory and also by eating, chewing, talking, or coughing. The intensity of the pain ranges from moderate to severe, although patients characteristically can touch themselves or talk while the pain is present. Pain is described as stabbing, burning, or electric shock-like. Cranial autonomic symptoms are very prominent in SUNCT syndrome, and characteristically both ipsilateral conjunctival injection and lacrimation accompany every attack. Rhinorrhea, eyelid edema, ptosis, miosis, and facial redness also can be present.
    SUNCT syndrome should be differentiated from essential trigeminal neuralgia (6, 13). Trigeminal neuralgia that involves only the territory of the first branch is very infrequent, occurring in only 1 to 4% of cases (18). In contrast to SUNCT syndrome, patients cannot talk or touch their face during attacks, and pain normally spreads over time to other trigeminal territories. Bouts of pain are shorter in trigeminal neuralgia than in SUNCT syndrome, and the typical refractory period after the pain attack is absent in patients with SUNCT syndrome. In addition, autonomic symptoms are uncommon in trigeminal neuralgia. Benoliel and Sharav (1) reviewed the occurrence of autonomic symptoms in a series of 22 patients diagnosed with trigeminal neuralgia. Six patients had lacrimation with the attacks, but none of them had first division involvement and all responded at some time to carbamazepine. Sjaastad et al. (18) also reviewed a group of patients with first-division trigeminal neuralgia and concluded that although autonomic symptoms, such as lacrimation, can be present in patients with trigeminal neuralgia, the combination of more than two autonomic symptoms is very rare. Autonomic symptoms are much more pronounced in SUNCT syndrome, and there is response at some time to carbamazepine in trigeminal neuralgia. However, some connection between these two pain syndromes may exist, because there are patients with trigeminal neuralgia in whom SUNCT syndrome eventually develops (3, 9). At presentation, the symptoms of our patient could have suggested the diagnosis of trigeminal neuralgia with first-division involvement. However, the presence of several prominent autonomic phenomena accompanying pain attacks, the duration of the attacks, and the fact that the patient could talk or touch herself during the pain bouts, together with the lack of response to therapeutic doses of carbamazepine and many other drugs, made the diagnosis of SUNCT syndrome almost certain.
   Other conditions such as cluster headache and migraine should be included in the differential diagnosis of this syndrome. Both have longer attack durations (minutes to hours), and cluster headache has low attack frequencies. A key diagnostic point is the presence in our patient of a clear-cut trigeminal trigger, which is characteristic of SUNCT syndrome. The lack of response of our patient to indomethacin and the aggravation of her symptoms by calcium channel antagonists, which normally are useful in migraine and migraine-like syndrome, also support the diagnosis (6).
   Many different pharmacological treatments have been tried on patients with SUNCT syndrome. This condition is refractory to treatments that are useful in other headache syndromes, such as carbamazepine, indomethacin, paracetamol, 5-hydroxytryptamine agonists, calcium channel antagonists, -blockers, prednisolone, phenytoin, and baclofen. Calcium channel antagonists are said even to worsen the pain symptoms (6). There are some reports of the efficacy of new antiepileptic drugs such as gabapentin or lamotrigine (4, 7, 12), but these are still scarce. Surgical treatment also has been tried. Some patients have responded to ablative procedures directed to the trigeminal ganglion such as balloon compression or glycerol rhizolysis (8, 16). Two patients have been treated successfully with microvascular decompression (5, 11), but another underwent the same procedure twice without success (2). In this last patient, an offending vascular structure was not found during surgery, but in one of the successfully treated patients, MRI did show a vascular compression before surgery, as occurred in our patient. Although surgical treatment should be reserved as the last therapeutic choice, when a vascular compression of the trigeminal root is identified, as occurred in our case, microvascular decompression can be effective in treating this syndrome. However, more experience is needed to determine the value of this technique in the treatment of this complex syndrome.
    Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome is a rare condition hardly ever encountered by those dealing with pain and may be confused with other pain types with autonomic origin. Therefore, its differential diagnosis and treatment are challenging and disputable. As was stated by the authors, making an interpretation about the treatment on the basis of only one case may be misleading. In planning the treatment modality, minimally invasive methods could be more appropriate as an initial step. This article is valuable because it shows that microvascular decompression may be applied in patients resistant to all treatment choices
   Yücel Kanpolat  Ankara, Turkey
    The authors have described an unusual syndrome that was successfully treated with microvascular decompression. This is a very rare phenomenon and is characterized by very short-lasting tic-like pain that affects the periorbital area and is associated with conjunctival injection, excessive tearing, rhinorrhea, and complaints of blurred vision. The latter symptoms clearly differentiate this syndrome from classical trigeminal neuralgia. This short-lasting neuralgiform pain is most commonly confused with cluster headache, although the prolonged character of cluster is a key diagnostic issue. There often is a trigger similar to that in trigeminal neuralgia; sometimes, the phenomenon is precipitated by exposure to bright light. Patients often have throat or ear pain, so confusion with glossopharyngeal neuralgia occurs.
    Medications commonly used for trigeminal neuralgia or other neuropathic pain sometimes help. Migraine therapies have been disappointing. The etiology is generally unknown; this is one of the few cases in which trigeminal root compression has been demonstrated. I have approached these patients as if they had trigeminal neuralgia; unfortunately, however, the medical therapies are not very effective. Failure to respond to carbamazepine is one of the diagnostic points of this syndrome, separating it from typical trigeminal neuralgia. Most direct surgical procedures on the ganglion have not been very successful, and only a few patients have been treated by microvascular decompression. The success of this surgery makes it likely that patients with from this rare syndrome can be identified by its clinical features and through failure of the usually successful medical treatments. Microvascular decompression may offer more successful therapy for patients who have failed virtually all other treatments.
    Donlin M. Long
    Baltimore, Maryland
Bès A et al Hypersensibilité dopaminergique dans la migraine: un test diagnostique ? La Nouvelle Presse Médicale1982; 11; 19
Blau JN Migraine postdromes: symptoms after attacks Cephalagia 1991 Nov; 11; 5; 229-31
Blin O, Azulay JP, Masson G, Aubrespy G, Serratrice G Apomorphine-induced yawning in migraine patients: enhanced responsiveness Clin Neuropharmacol 1991 Feb; 14; 1; 91-5
Cerbo R, Barbanti P, Buzzi MG, Fabbrini G, Brusa L, Roberti C, Zanette E, Lenzi GL Dopamine hypersensitivity in migraine: role of the apomorphine test Clin Neuropharmacol 1997 Feb; 20; 1; 36-4
Del Bene E, M Poggioni Video assessment of yawning induced by sublingual apomorphine in migraine Headache 1994; 34; 9; 536-8
Del Zompo M Dopamine Hypersensitivity in Migraine: Role in Apomorphine Syncope Headache 1995; 35; 222-224
Del Zompo M Association between dopamine receptor genes and migraine without aura in a Sardinian sample Neurology 1998; 51; 3; 781-6
Fanciullacci M, Alessandri M, Del Rosso A Dopamine involvement in the migraine attack. Funct Neurol 2000; 15 Suppl 3:171-81
Heinrichs L Linking olfaction with nausea and vomiting of pregnancy, recurrent abortion, hyperemesis gravidarum, and migraine headache Headache 2003; 43; 3; 304-5
Jacome D Compulsive yawning as migraine premonitory symptom Cephalalgia 2001 Jun; 21; 5; 623-5
Jacome D Primary yawning headache Cephalalgia 2001 Jul; 21; 6; 697-699
James MF,Smith J M et al Cortical spreading depression and migraine: new insights from imaging? Trends in Neurosciences May 2001; vol 24; n 5; p 266-271
Loder A What is the evolutionary advantage of migraine ? Cephalalgia 2002; 22; 624-632
Peres MF et al Hypothalamic involvement in chronic migraine J Neurol Neurosurg Psychiatry 2001; 71; 747-751
Peroutka SJ Dopamine and migraine Neurology 1997 Sep; 49; 3; :650-6
Pietrobon D, J Sriessnig Neurobiology of migraine Nature Neurosciences Reviews may 2003; vol 4; n°5; p386-398
Sabatini U et al Migraine attacks induced by subcutaneous apomorphine in two migrainous parkinsonian patients Clinical Neuropharmacology1990; 13; 3; 264-267
Szechtman H et al Sensitization and Tolerance to Apomorphine in Men: Yawning, Growth Hormone, Nausea, and Hyperthermia Psychiatry Research 1988, 23, 245-255
headphone neuralgia Skelton A
Glossopharyngeal neuralgia and MS A Minagar
Paroxysmal kinesigenic dystonia associated with a medullary lesion DE Riley
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing syndrome treated with microvascular decompression of the trigeminal nerve A Lagares