Chronic Paroxysmal Hemicrania-Tic Syndrome
Address all correspondence to Dr. A. Martínez-Salio, Department of Neurology, Hospital Universitario 12 de Octubre, Ctra de Andalucía km 5,400, 28041 Madrid, Spain.
The coexistence of chronic paroxysmal hemicrania and trigeminal neuralgia is called chronic paroxysmal hemicrania-tic syndrome. We describe the case of a man who has suffered both types of pain occurring synchronously but with different localization on the ipsilateral side. The pain attacks could be abolished with indomethacin and carbamazepine. To the best of our knowledge, this is the third case to be reported, the first in the male sex. We review this new disorder and discuss the pathophysiology.
chronic paroxysmal hemicrania
chronic paroxysmal hemicrania-tic syndrome
episodic paroxysmal hemicrania
Cluster-tic syndrome is a disorder characterized by the association of cluster headache and trigeminal neuralgia. The disorder combines three types of pain attacks. One resembles trigeminal neuralgia, the second resembles cluster headache, and the third is mixed. The nature of this disorder is usually unknown, and its course is either episodic or chronic. Carbamazepine together with medication for cluster headache is the treatment of choice. 1–4
Chronic paroxysmal hemicrania (CPH), first described by Sjaastad and Dale, 5 is the other headache of the cluster headache category. The prevalence of CPH was estimated to be about 2% of that of cluster headache. 6,7 The coexistence of both CPH and trigeminal neuralgia (tic doloureux), analogous to the cluster-tic syndrome, is called chronic paroxysmal hemicrania-tic syndrome (CPH-tic). Only two cases have been described in the literature to date. 8,9
We present a patient with features of both CPH and trigeminal neuralgia, and review the literature.
A 52-year-old man came to our attention at the outpatient service, presenting a 2-month history of headache. The pain was described as severe, sharp, or stabbing, and was located behind the right eye and in the temporal region. Attacks would recur five to eight times a day and last for 2 to 15 minutes. During the attacks, there was ipsilateral lacrimation, conjunctival injection, nasal congestion, and rhinorrhea but no eyelid edema or ptosis. The attacks occurred both during sleep and throughout the day, usually in the evening. Previous treatments with paracetamol, aspirin, and propranolol provided no relief. His past medical history was unremarkable. The physical and neurological examinations in the attack-free periods were absolutely normal. Routine hematology and blood chemistry studies were unremarkable, as was an MRI of the brain. He was diagnosed as having CPH. Treatment with indomethacin, 25 mg three times a day, resulted in an immediate and complete response. The patient tried to withdraw the treatment after 1 month, but the pain came back and disappeared again with indomethacin. Finally, the treatment was stopped 6 months later, and the patient remained asymptomatic.
Four months later, he developed another pain on the same side as his chronic paroxysmal headache attacks. The pain was severe in intensity and was described as brief electric shocklike pains, spreading from the jaw to the right ear, confined to the third branch of the fifth cranial nerve, lasting for only seconds at the most and was evoked by touching the affected area, by talking, and by chewing. There was no conjunctival injection, lacrimation, rhinorrhea, or nasal congestion during the attacks. Treatment with indomethacin provided no relief. His general physical and neurological examination only revealed a trigger spot on the lower lip. The pain was diagnosed as trigeminal neuralgia affecting the mandibular branch of the fifth cranial nerve. He was treated with carbamazepine, 200 mg three times a day, which resulted in a complete response in 24 hours. One month later, he discontinued the treatment but the pain recurred, and he resumed carbamazepine.
Two months after the beginning of the trigeminal neuralgia, on treatment with carbamazepine, the first headache reappeared. Attacks would recur five to seven times per day and last for 5 to 15 minutes. The pain was excruciating, stabbing, located in the right ocular and temporal region, and associated with ipsilateral lacrimation, nasal blockage, conjunctival injection, and rhinorrhea. Treatment with indomethacin, 25 mg three times a day, again resulted in an immediate and complete response.
One month later, he again discontinued indomethacin but without recurrences. The carbamazepine was also discontinued slowly. After a 3-month follow-up, the patient continues to be asymptomatic without treatment.
The patient we report suffered from two types of painful attacks. The second type is clearly consistent with trigeminal neuralgia affecting the mandibular division. The first type has most of the features of CPH, according to the International Headache Society (IHS) diagnostic criteria for this syndrome. 10 The criteria for CPH include at least 50 attacks of severe unilateral, orbital, supraorbital and/or temporal pain always on the same side lasting 2 to 45 minutes, with an attack frequency of more than five a day for more than half the time (periods with lower attack frequency may occur). The pain is associated with at least one of the following signs/symptoms on the painful side: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, ptosis, and eyelid edema. There is an absolute effectiveness of indomethacin (usually 150 mg/day or less). The history, examination, and appropriate investigations should not suggest another disorder.
Our patient has some atypical features. He is a man, and this syndrome mainly affects women, with a male to female ratio of 1:3, but sex preponderance, once considered to be an issue in CPH, has not been made a criterion. 6 The duration and frequency of attacks are low, but they are in accordance with the IHS diagnostic criteria. 10 However, the two remissions, 6 months and then 1 month, off medication are problematic. This patient may have a remitting form of CPH, as suggested by Sjaastad and Antonaci. 11 Alternatively, the patient could fit the criteria for episodic paroxysmal hemicrania (EPH) which, according to the IHS definition, differs from CPH as follows: an attack frequency of three or more a day with each attack lasting 1 to 30 minutes and clear intervals between bouts of attacks that may last from months to years. 12–14 Since transformations from EPH to CPH have been described, 6 some authors consider EPH to be a prechronic stage of CPH. However, EPH has been reported to remain episodic for decades, 15 and conversion from the chronic to episodic phase has been reported. 16 Some authors consider EPH to be an episodic variant of CPH, the remitting form. The distinction may be one of arbitrary terminology only (remitting CPH versus EPH) since there are no biological markers or diagnostic studies to distinguish the two.
The first headache does not fit the IHS criteria for cluster headache or hemicrania continua. Although the attack frequency is consistent with the 1 to 8 per day for cluster headache, the distinctions are shorter duration (cluster's duration is 15 to 180 minutes) and the absolute responsiveness to indomethacin. Hemicrania continua contrasts with our patient's pain by the presence of a constant, low-level, baseline headache. The painful exacerbations of hemicrania continua tend to be less severe and the ipsilateral autonomic features are also less pronounced than in our patient. Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing, another rare disorder, differs from CPH because of an attack duration lasting only 15 to 120 seconds and an attack frequency from 3 to 100 per day. Our patient's first pain is distinguished from trigeminal neuralgia because of the longer duration of attacks and associated autonomic features.
We describe here what we believe to be the third case of CPH-tic. The first case reported is that of Hannerz in 1993. 8 His patient had a chronic form with both types of pain occurring asynchronously and with different localization; CPH attacks in the orbital region and tic paroxysms in the ipsilateral V3 and V2 territories. The second patient, another woman, had co-localized attacks with features of both V1 tic and CPH appearing in a synchronous fashion and with a remitting pattern. 9 Our patient had attacks of both types of pain occurring synchronously as did the second patient, but with different localization on the ipsilateral side as in the first patient.
The pathophysiological mechanism responsible for the pain in CPH-tic remains unknown. A single lesion affecting the trigeminal sensory pathway is probably the cause, as in cluster-tic syndrome. The appearance of distinct pains indicates that different trigeminal fibers are affected. Involvement of small myelinated fibers could be responsible for neuralgic attacks, while disturbance of unmyelinated fibers could cause the CPH.
The location of the lesion in the trigeminal pathway is also unknown, but the supposed origin of CPH attacks is the cavernous sinus. When the extracavernous branch of the trigeminal nerve (V3) is involved, as in our patient, tic and CPH attacks could occur independently and in different localizations, on the same symptomatic side. Caminero et al, 9 who called this syndrome CPH-tic V3 in opposition to CPH-tic V1, suggested this hypothesis which may explain the different response to treatment.
Another possibility is a posterior fossa lesion affecting the trigeminal sensory root. This hypothesis has been suggested in the cluster-tic syndrome. Vascular compression of the sensory root has been found in several patients with cluster-tic, 17,18 and a posterior fossa cholesteatoma causing trigeminal and glossopharyngeal neuralgia pain, as well as cluster headache, has also been reported. 19 Nevertheless, the autonomic signs cannot be explained by isolated posterior fossa trigeminal sensory root compression, but the CPH pain could be precipitated by the neuralgic pain. The mechanism could be similar to that previously observed in some patients with CPH, who report that attacks may be precipitated by bending or rotating the head, exerting pressure on the transverse processes of C4-C5, the C2 root, or the ipsilateral greater occipital nerve. Furthermore, authors have suggested that both CPH and trigeminal neuralgia may be due to a primary central triggering mechanism in the brain stem and a secondary involvement of peripheral factors probably mediated by neurogenic impulses.
It remains unclear if CPH-tic syndrome is a new, distinct entity or whether it represents the coexistence of two different diseases, where one of them triggers the other. Further reports will help to clarify this question and will lead to a better understanding of the pathophysiology of these entities.