Cluster Headache, SUNCT, and SUNA.

OBJECTIVE: This article reviews the epidemiology, clinical features, differential diagnosis, pathophysiology, and management of three types of trigeminal autonomic cephalalgias: cluster headache (the most common), short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). LATEST DEVELOPMENTS: The first-line treatments for trigeminal autonomic cephalalgias have not changed in recent years: cluster headache is managed with oxygen, triptans, and verapamil, and SUNCT and SUNA are managed with lamotrigine. However, new successful clinical trials of high-dose prednisone, high-dose galcanezumab, and occipital nerve stimulation provide additional options for patients with cluster headache. Furthermore, new genetic and imaging tests in patients with cluster headache hold promise for a better understanding of its pathophysiology. ESSENTIAL POINTS: The trigeminal autonomic cephalalgias are a group of diseases that appear similar to each other and other headache disorders but have important differences. Proper diagnosis is crucial for proper treatment.

SUNCT, SUNA and short-lasting unilateral neuralgiform headache attacks: Debates and an update.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks (SUNHA) have the features of both short-lasting unilateral neuralgiform pain, such as trigeminal neuralgia or stabbing headache, and associated trigeminal autonomic symptoms, such as paroxysmal hemicrania or cluster headache. Recognizing and adequately treating SUNHA is essential but current treatment methods are ineffective in treating SUNHA. METHODS: We reviewed the changes in the concept of short-lasting unilateral neuralgiform headache attacks and provide a narrative review of the current medical and surgical treatment options, from the first choice of treatment for patients to treatments for selective intractable cases. RESULTS: Unlike the initial impression of an intractable primary headache disorder affecting older men, SUNHA affects both sexes throughout their lifespan. One striking feature of SUNHA is that the attacks are triggered by cutaneous or intraoral stimulation. The efficacy of conventional treatments is disappointing and challenging, and preventive therapy is the mainstay of treatment because of highly frequent attacks of a very brief duration. Amongst them, lamotrigine is effective in approximately two-third of the patients with SUNHA, and intravenous lidocaine is essential for the management of acute exacerbation of intractable pain. Topiramate, oxcarbazepine and gabapentin are considered good secondary options for SUNHA, and botulinum toxin can be used in selective cases. Neurovascular compression is commonly observed in SUNHA, and surgical approaches, such as neurovascular compression, have been reported to be effective for intractable cases. CONCLUSIONS: Recent advances in the understanding of SUNHA have improved the recognition and treatment approaches for this unique condition.

Efficacy of Occipital Nerve Stimulation in Trigeminal Autonomic Cephalalgias: A Systematic Review.

BACKGROUND: Trigeminal autonomic cephalalgias (TACs) are a group of highly disabling primary headache disorders. Although pharmacological treatments exist, they are not always effective or well tolerated. Occipital nerve stimulation (ONS) is a potentially effective surgical treatment. OBJECTIVE: To perform a systematic review of the efficacy of ONS in treating TACs. METHODS: A systematic review was performed using Medline, Embase, and Cochrane databases. Primary outcomes were reduction in headache intensity, duration, and frequency. Secondary outcomes included adverse event rate and reduction in medication use. Because of large differences in outcome measures, data for patients suffering from short-lasting, unilateral, and neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and cranial autonomic symptoms (SUNA) were reported separately. Risk of bias was assessed using the NIH Quality Assessment Tools. RESULTS: A total of 417 patients from 14 published papers were included in the analysis, of which 15 patients were in the SUNCT/SUNA cohort. The mean reduction in headache intensity and duration was 26.2% and 31.4%, respectively. There was a mean reduction in headache frequency of 50%, as well as a 61.2% reduction in the use of abortive medications and a 31.1% reduction in the use of prophylactic medications. In the SUNCT/SUNA cohort, the mean decrease in headache intensity and duration was 56.8% and 42.8%. The overall responder rate, defined as a >50% reduction in attack frequency, was 60.8% for the non-SUNCT/non-SUNA cohort and 66.7% for the SUNCT/SUNA cohort. Adverse events requiring repeat surgery were reported in 33% of cases. Risk of bias assessment suggests that articles included in this review had reasonable internal validity. CONCLUSION: ONS may be an effective surgical treatment for approximately two thirds of patients with medically refractory TACs.

Trigeminal Autonomic Cephalalgias and Neuralgias in Children and Adolescents: a Narrative Review.

PURPOSE OF REVIEW: To summarize the available literature as well as the authors' experience on trigeminal autonomic cephalalgias (TACs) and cranial neuralgias in children and adolescents. RECENT FINDINGS: While TACs and cranial neuralgias are rare in children, several recent case series have been published. TACs in children share most of the clinical features of TACs in adults. However, there are many reported cases with clinical features which overlap more than one diagnosis, suggesting that TACs may be less differentiated in youth. Indomethacin-responsive cases of cluster headache and SUNCT/SUNA have been reported in children, whereas in adults indomethacin is usually reserved for paroxysmal hemicrania and hemicrania continua. Neuralgias appear to be rare in children. Clinical features are often similar to adult cases, though clinicians should maintain a high index of suspicion for underlying causes.

Central generators of migraine and autonomic cephalalgias as targets for personalized pain management: Translational links.

BACKGROUND AND OBJECTIVE: Migraine oscillates between different states in association with internal homeostatic functions and biological rhythms that become more easily dysregulated in genetically susceptible individuals. Clinical and pre-clinical data on migraine pathophysiology support a primary role of the central nervous system (CNS) through 'dysexcitability' of certain brain networks, and a critical contribution of the peripheral sensory and autonomic signalling from the intracranial meningeal innervation. This review focuses on the most relevant back and forward translational studies devoted to the assessment of CNS dysfunctions involved in primary headaches and discusses the role they play in rendering the brain susceptible to headache states. METHODS AND RESULTS: We collected a body of scientific literature from human and animal investigations that provide a compelling perspective on the anatomical and functional underpinnings of the CNS in migraine and trigeminal autonomic cephalalgias. We focus on medullary, hypothalamic and corticofugal modulation mechanisms that represent strategic neural substrates for elucidating the links between trigeminovascular maladaptive states, migraine triggering and the temporal phenotype of the disease. CONCLUSION: It is argued that a better understanding of homeostatic dysfunctional states appears fundamental and may benefit the development of personalized therapeutic approaches for improving clinical outcomes in primary headache disorders. SIGNIFICANCE: This review focuses on the most relevant back and forward translational studies showing the crucial role of top-down brain modulation in triggering and maintaining primary headache states and how these central dysfunctions may interact with personalized pain management strategies.

Symptomatic trigeminal autonomic cephalalgias in neuromyelitis optica spectrum disorders.

BACKGROUND: The pathophysiology of trigeminal autonomic cephalalgias (TACs) is poorly understood at present. Symptomatic TACs are rarely reported in neuromyelitis optica spectrum disorders (NMOSD). To better clarify this distinct clinical manifestation in NMOSD and to investigate its possible pathophysiology, we reviewed articles describing such cases including our own case. METHODS: We performed a search of all clinical studies of TACs in NMOSD published up to September 1st, 2022. We put no restrictions on the year of English publication in our search. The following keywords were searched: trigeminal autonomic cephalalgias, cluster headache, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), hemicrania continua, paroxysmal hemicrania, neuromyelitis optica, neuromyelitis optica spectrum disorder, Devic's disease. RESULT: We reviewed six cases (five published reports and our own case study) that fulfilled the diagnosis of NMOSD and TACs. Four of them were SUNCT, one was SUNA, and one was paroxysmal hemicrania. In three of these cases, headache was the initial sole manifestation. Only one case had a good response to routine TACs' treatment. All these patients had lesions in the medulla oblongata and cervical cord. Three cases' TACs were side-locked, and two of them had a left dorsolateral medulla oblongata lesion that corresponded with the left side TACs, while three cases' headaches happened on either side of the head. The phenomenon could be explained by the activation of trigeminal-autonomic reflex and ephaptic coupling. CONCLUSION: TACs could be the initial sole brainstem manifestation of NMOSD. An underlying cause for SUNCT/SUNA should be considered, especially if there is a limited response to anti-epileptic medication. The activation of trigeminal-autonomic reflex and ephaptic coupling might be the underlying mechanism of symptomatic TACs in NMOSD.

Management of Trigeminal Autonomic Cephalalgias Including Chronic Cluster: A Review.

Importance: Trigeminal autonomic cephalalgias (TACs) comprise a unique collection of primary headache disorders characterized by moderate or severe unilateral pain, localized in in the area of distribution of the first branch of the trigeminal nerve, accompanied by cranial autonomic symptoms and signs. Most TACs are rare diseases, which hampers the possibility of performing randomized clinical trials and large studies. Therefore, knowledge of treatment efficacy must be based only on observational studies, rare disease registries, and case reports, where real-world data and evidence play an important role in health care decisions. Observations: Chronic cluster headache is the most common of these disorders, and the literature offers some evidence from randomized clinical trials to support the use of pharmacologic and neurostimulation treatments. Galcanezumab, a monoclonal antibody targeting the calcitonin gene-related peptide, was not effective at 3 months in a randomized clinical trial but showed efficacy at 12 months in a large case series. For the other TACs (ie, paroxysmal hemicrania, hemicrania continua, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms), only case reports and case series are available to guide physicians in everyday management. Conclusions and Relevance: The accumulation of epidemiologic, pathophysiologic, natural history knowledge, and data from case series and small controlled trials, especially over the past 20 years from investigators around the world, has added to the previously limited evidence and has helped advance and inform the treatment approach to rare TACs, which can be extremely challenging for clinicians.

Recent Advances and Updates in Trigeminal Autonomic Cephalalgias.

Trigeminal autonomic cephalalgias (TACs) are discrete primary headache disorders, characterized by severe unilateral head pain, typically trigeminal distribution, with ipsilateral cranial autonomic symptoms. The conditions within this group are hemicrania continua, cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing and short-lasting unilateral neuralgiform headache with autonomic symptoms. Several advances have been made in understanding the pathogenesis and evolving treatment options in TACs. This review will outline the advances and updates in each TAC.

[Trigeminal Autonomic Cephalgias]. / Trigeminoautonome Kopfschmerzen.

Compared with migraine and tension-type headache, trigeminal autonomic cephalgias (TAC) are rare, but the resulting significant impairment and the not irrelevant prevalence (e. g., cluster headache 0.1%) make TACs important diagnoses. Unfortunately, the correct diagnosis is often delayed. This article provides an overview of the diagnostic approach and therapeutic options in TACs.

[Autonomic Disorders in Trigeminal Autonomic Cephalalgias: An Update].

The term trigeminal autonomic cephalalgias (TACs) was introduced in the 2nd edition of the International Classification of Headache Disorders, and has been retained in the 3rd edition. TACs include cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks, hemicrania continua, and probable trigeminal autonomic cephalalgia. Headaches classified as TACs share clinical features with unilateral headache and usually present with prominent lateralized and ipsilateral cranial parasympathetic autonomic features, including the following: (1) conjunctival injection and/or lacrimation, (2) nasal congestion and/or rhinorrhea, (3) eyelid edema, (4) forehead and facial sweating, (5) miosis and/or ptosis. "Clinical Practice Guideline for Headache 2021 (Japanese version)" will serve as a useful aid for diagnosis and treatment of TACs. Currently, hypothalamic activation, activation of the trigeminal-autonomic nerve reflex, internal carotid artery dilatation, and the action of some neuropeptides are implicated as pathophysiological mechanisms underlying TACs; however, it is not unequivocal. Further studies are warranted to gain deeper insight into several unclear aspects associated with TACs.

Cluster Headache and Other Trigeminal Autonomic Cephalalgias.

PURPOSE OF REVIEW: The trigeminal autonomic cephalalgias (TACs) are relatively rare, but they represent a distinct set of syndromes that are important to recognize. Despite their unique features, TACs often go undiagnosed or misdiagnosed for several years, leading to unnecessary pain and suffering. A significant proportion of TAC presentations may have secondary causes. RECENT FINDINGS: The underlying pathophysiology of TACs is likely rooted in hypothalamic dysfunction and derangements in the interplay of circuitry involving trigeminovascular, trigeminocervical, trigeminoautonomic, circadian, and nociceptive systems. Recent therapeutic advancements include a better understanding of how to use older therapies more effectively and the identification of new approaches. SUMMARY: TAC syndromes are rare but important to recognize because of their debilitating nature and greater likelihood for having potentially serious underlying causes. Although treatment options have remained somewhat limited, scientific inquiry is continually advancing our understanding of these syndromes and how best to manage them.

Paroxysmal Hemicrania: An Update.

Paroxysmal Hemicrania (PH) is classified under trigeminal autonomic cephalalgia (TAC) as per the International Classification of Headache Disorders (ICHD). Since the first description by 0ttar Sjaastad and Inge Dale in 1974, PH has been reported by many authors. A greater understanding of PH phenotype and pathophysiology has resulted in the evolution of its diagnostic criteria, and management. We tabulated major case series of PH to describe the epidemiology, clinical features and recent updates of PH. PH is a rare headache characterized by daily, multiple paroxysms of unilateral, short-lasting (mean duration <20 minutes), side-locked headache in the distribution of ophthalmic division of trigeminal nerve with associated profound cranial autonomic symptoms. Recent ICHD classification added "restlessness" to the criteria for PH. Pain should completely respond to indomethacin to fulfil the diagnostic criteria of PH. PH should be differentiated from cluster headache, SUNCT/SUNA, and other short-lasting side-locked headaches. Trigeminal afferents possibly produce pain in PH and trigeminal-autonomic reflex explains the occurrence of autonomic features. Recently, a "permissive" central role of the hypothalamus is unveiled based on functional imaging studies. Other Cox-2 inhibitors, topiramate, calcium-channel blockers, epicranial nerve blocks have been shown to improve headache in some patients of PH who cannot tolerate indomethacin. Hypothalamic deep brain stimulation has been used in treatment-refractory cases.

SUNCT and SUNA: An Update.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA) are rare and disabling primary headache disorders that are subtypes of Short-lasting unilateral neuralgiform headache attacks (SUNHA). AIM: The aim of this narrative review was to provide a comprehensive update on headache phenotype, pathophysiology, and various treatment options available for SUNCT and SUNA. METHODS: References for this review were identified by searches of articles published in the English language in PubMed between 1978 and October 2020 using "short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)", "short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA)", "short-lasting unilateral neuralgiform headache attacks (SUNHA)", "trigeminal autonomic cephalalgias" as keywords in various combinations. RESULTS: Of a potential 1103 articles, seven case series describing clinical characteristics of SUNCT/SUNA patients were identified for this review. For symptomatic/secondary SUNCT/SUNA, 53 individual case reports, and one case series were reviewed. One placebo-controlled trial and 11 open-label case series that evaluated various medical and surgical treatments in SUNCT/SUNA were also reviewed. Available literature suggests that SUNCT and SUNA are subtypes of the same disorder characterized by severe side locked short duration headache with ipsilateral prominent cranial autonomic symptoms and signs. Pathophysiology may involve both peripheral and central mechanisms. Lamotrigine is the most effective preventive therapy while intravenous lidocaine is the most efficacious drug as transitional therapy for severe disabling attacks. Surgical options including microvascular decompression in those having neurovascular conflict, occipital nerve stimulation, and hypothalamic deep brain stimulation can be alternative treatment options for medically refractory patients.

SUNCT/SUNA in children and adolescents: Application of ICHD-3 criteria and treatment response: Case series of 13 SUNCT/SUNA pediatric cases.

INTRODUCTION: Trigeminal autonomic cephalalgias (TACs) are characterized by paroxysmal attacks of unilateral primary headaches associated with ipsilateral craniofacial autonomic symptoms. In this pediatric case series, 13 cases of short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT)/short-lasting unilateral neuralgiform headache with autonomic symptoms (SUNA), including children ages 3-18 years, are discussed. This paper reviews the application of International Classification of Headache Disorders, 3rd edition (ICHD-3) criteria especially in children presenting with SUNCT or SUNA. This is the largest pediatric case series of SUNCT/SUNA reported in the literature. BACKGROUND: Trigeminal autonomic cephalalgias are rare in children and adolescents, with SUNCT/SUNA having the least reported cases. We will discuss the application of ICHD-3 criteria to diagnose SUNCT/SUNA in children and review overlapping cases and their response to different treatment options including indomethacin, which is typically reserved for specific subtypes of TACs; for example, paroxysmal hemicrania. CONCLUSION: This case series presents a unique opportunity to aid in the diagnosis and treatment of similar pediatric cases in the future. It helps us to broaden the ICHD-3 criteria to diagnose and treat different overlapping trigeminal autonomic cephalalgia cases in children.

[Cluster headaches-proven and novel approaches : A critical evaluation of treatment studies]. / Clusterkopfschmerzen ­ Bewährtes und Neues : Therapiestudien kritisch bewertet.

Trigeminal autonomic cephalalgias are characterized by unilateral headaches accompanied by cranial autonomic symptoms and a sense of restlessness. Clinical trials have been conducted on acute therapy and prophylaxis of cluster headache in recent years. Since the other trigeminal autonomic cephalalgias are rare, it is virtually impossible to conduct controlled clinical trials for their investigation. Recent trials provide data on the efficacy of prednisolone, galcanezumab, fremanzeumab and noninvasive vagal nerve stimulation. Methodological difficulties arise in terms of patient recruitment for clinical trials, clinical endpoints and potential differences in the pathophysiology of episodic and chronic cluster headache.

[Clinical Aspects of Short-Lasting Unilateral Neuralgiform Headache Attacks].

Short-lasting unilateral neuralgiform headache attacks (SUNHA), within the group of trigeminal autonomic cephalalgias, are characterized by repetitive, brief and severe attacks. The SUNHA includes short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA). SUNCT is diagnosed if both conjunctival injection and tearing occur ipsilateral to the pain, whereas SUNA is diagnosed if only one, or neither, of conjunctival injection and tearing occurs. Previously considered to be rare and rather refractory to treatment, there is increasing awareness of this syndrome and the therapeutic possibilities. During the worst periods, intravenous lidocaine may decrease the intensity of SUNHA. Lamotrigine is the most-effective prophylactic treatment. For medically intractable SUNHA, several surgical approaches, i.e., microvascular decompression of the trigeminal nerve and neuromodulation, have been tried. There is considerable clinical, therapeutic and radiological overlap between SUNCT, SUNA, and trigeminal neuralgia. Despite being considered distinct conditions, the emerging evidence suggests a broader nosological concept of SUNCT, SUNA, and trigeminal neuralgia; these conditions may constitute a continuum of the same disorder, rather than separate clinical entities. This article discusses the clinical aspects of the syndrome, including pathophysiology, differential diagnosis, radiological imaging and treatment. (Received May 26, 2020; Accepted June 16, 2020; Published November 1, 2020).

[Cluster headache and other trigeminal autonomic cephalalgias]. / Clusterhoofdpijn en andere trigeminale autonome cefalalgieën.

Trigeminal autonomic cephalalgias (TACs) are a group of 4 different primary headache syndromes that have a lot of pathophysiological and clinical features in common. The 4 different TACs are: cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks and hemicrania continua. TACs are characterized by frequent, strictly unilateral, (very) intense headache attacks with ipsilateral cranial autonomic symptoms or intrinsic restlessness or both. A distinction can be made between the 4 TACs on the basis of the duration and frequency of the headache attacks. The treatment of cluster headache consists of an acute treatment and a maintenance treatment. Headache attacks in the context of paroxysmal hemicrania and hemicrania continua (almost) always respond to treatment with indomethacin. More and more neuromodulation therapies are becoming available, such as vagus nerve stimulation, stimulation and blocking of the sphenopalatine ganglion, stimulation and blocking of the occipital nerve and deep brain stimulation.

Cluster headache and TACs: state of the art.

Cluster headache (CH), paroxysmal hemicrania (PH), short-lasting unilateral neuralgiform headache attacks (including SUNCT and SUNA), and hemicrania continua (HC) compose the group of trigeminal autonomic cephalalgias (TACs). Here, we review the recent advances in the field and summarize the current knowledge about the origin of these headaches. Similar to the other primary headaches, the pathogenesis is still much obscure. However, advances are being made in both animal models and humans studies. Three structures clearly appear to be crucial in the pathophysiology of TACs: the trigeminal nerve, the facial parasympathetic system, and the hypothalamus. The physiologic and pathologic functioning of each of these elements and their interactions is being progressively clarified, but critical questions are still open.

Sphenopalatine Ganglion Pulsed Radiofrequency for the Treatment of Refractory Chronic SUNCT and SUNA: A Prospective Case Series.

OBJECTIVES: We aimed to evaluate the safety and effectiveness of sphenopalatine ganglion pulsed radiofrequency (SPG-PRF) for the treatment of patients with refractory chronic short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and with cranial autonomic symptoms (SUNA). BACKGROUND: SPG-PRF is a minimally invasive, non-neurodestructive procedure already tested in refractory chronic cluster headache with mixed outcomes. However, no data have been produced in SUNCT/SUNA. METHODS: This was a prospective clinical audit of outcomes. Consecutive patients with chronic SUNCT/SUNA refractory to medical treatments and treated with SPG-PRF, were included in the analysis. The SPG-PRF was performed percutaneously via a lateral, infra-zygomatic approach. Responders were defined as patients with a reduction in number and/or severity of headache episodes by ≥30% for ≥3 months. Responders to the first procedure were offered to have the treatment repeated. RESULTS: Nine patients (6 female) were analyzed. After a median follow-up of 30 months (range 2-30), 7 patients were considered responders to the treatment (77.8%) for a median of 6 months (range: 4-10), 1 patient obtained 40% improvement for 2 months, and 1 patient did experience any improvement. No procedure-related immediate or delayed side effects were reported. Three patients (33.3%) experienced a worsening of the head pain for 2-4 weeks immediately after the procedure. Four responders had SPG-PRF repeated; a reproducible response was obtained in two of them. CONCLUSIONS: In our small series of patients with refractory chronic SUNCT/SUNA, SPG-PRF was a safe and effective treatment modality. The potential reproducible positive effect of subsequent treatments may prevent or delay the use of more invasive and costly interventions for at least a proportion of these patients.

Facial presentations of migraine, TACs, and other paroxysmal facial pain syndromes.

OBJECTIVE: To assess the prevalence of facial pain (V2 and/or V3) presentations among nearly 3,000 patients with headache treated in a university tertiary care center. METHODS: Between 2010 and 2018, we routinely assessed the prevalence of facial pain presentations of all patients with primary headaches. RESULTS: Of 2,912 patient datasets, 291 patients reported facial pain either as an independent or as an additional symptom. Among patients with migraine, 2.3% (44 of 1,935) reported a facial involvement, most commonly in V2. Of these, 18 patients (40.9%) experienced the pain predominantly in the face. In patients with cluster headache, 14.8% (42 of 283) reported a facial involvement, of which 31.0% perceived the pain predominantly in the face. A facial involvement was seen in 45.0% of patients with paroxysmal hemicrania (9 of 20), 21.4% of patients with hemicrania continua (9 of 42), and 20.0% of patients with short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (3 of 15). In addition, we present 6 patients who reported a constant side-locked facial pain with superseded well-defined facial pain attacks of 10- to 30-minute duration that appeared several times per day. CONCLUSION: Our data suggest that a facial involvement in primary headaches is infrequent but not uncommon. A sole facial presentation of primary headache symptomatology seems to be exceptionally rare. We describe 3 different types of facial pain involvement and, in this context, distinguish patients with paroxysmal orofacial pain syndromes that have not been previously described. These patients may represent a new entity that could tentatively be called constant unilateral facial pain with added attacks.