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Strategies for the treatment of autonomic trigeminal cephalalgias.

Trigeminal autonomic cephalalgias (TACs) are a group of primary headache syndromes characterised by two main clinical characteristics: pain and oculofacial autonomic phenomena. Three headache forms are grouped as TACs: cluster headache (CH), paroxysmal hemicrania (PH) and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). These are distinguished mainly on the basis of attack duration. It lasts from 15 to 180 min in CH, from 2 to 30 min in PH and from 5 to 240 s in SUNCT. The most effective drug preventative in PH is indomethacin even if in few cases other non-steroidal anti-inflammatory drugs have been reported to be effective. SUNCT is commonly described as drug resistant. Recent studies report that lamotrigin may be the drug of choice for SUNCT.

Adrenal Cortex Hormones↗

Trigeminal autonomic cephalalgias. Pathophysiology and classification.

Trigeminal Autonomic Cephalalgias (TACs) is a grouping of headache syndromes that includes cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). They are recognized clinically by their episodic, stereotypic attack profile and very often prominent cranial autonomic symptoms, such as lacrimation, conjunctival injection or rhinorrhea. They involve afferent activation of the trigeminal innervation of intracranial pain-producing structures, or the perception of that activation, and reflex activation of the facial, seventh cranial, nerve outflow pathway. This excess reflex trigeminal-autonomic activation seems to be permitted by dysfunction in the brain, specifically in the posterior hypothalamic gray matter. Understanding the anatomy and physiology of these disorders has greatly facilitated their management and the development of exciting new strategies such as neuromodulatory approaches to the management of the more intractable cases.

Cluster Headache↗

EFNS guidelines on the treatment of cluster headache and other trigeminal-autonomic cephalalgias.

Cluster headache and the other trigeminal-autonomic cephalalgias [paroxysmal hemicrania, short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome] are rare but very disabling conditions with a major impact on the patient's quality of life. The objective of this study was to give evidence-based recommendations for the treatment of these headache disorders based on a literature search and consensus amongst a panel of experts. All available medical reference systems were screened for any kind of studies on cluster headache, paroxysmal hemicrania and SUNCT syndrome. The findings in these studies were evaluated according to the recommendations of the European Federation of Neurological Societies resulting in level A, B or C recommendations and good practice points. For the acute treatment of cluster headache attacks, oxygen (100%) with a flow of at least 7 l/min over 15 min and 6 mg subcutaneous sumatriptan are drugs of first choice. Prophylaxis of cluster headache should be performed with verapamil at a daily dose of at least 240 mg (maximum dose depends on efficacy or tolerability). Although no class I or II trials are available, steroids are clearly effective in cluster headache. Therefore, the use of at least 100 mg methylprednisone (or equivalent corticosteroid) given orally or at up to 500 mg i.v. per day over 5 days (then tapering down) is recommended. Methysergide, lithium and topiramate are recommended as alternative treatments. Surgical procedures, although in part promising, require further scientific evaluation. For paroxysmal hemicranias, indomethacin at a daily dose of up to 225 mg is the drug of choice. For treatment of SUNCT syndrome, large series suggest that lamotrigine is the most effective preventive agent, with topiramate and gabapentin also being useful. Intravenous lidocaine may also be helpful as an acute therapy when patients are extremely distressed and disabled by frequent attacks.

Advisory Committees↗

Trigeminal autonomic cephalalgias: from pathophysiology to clinical aspects.

The strictly unilateral headaches, more commonly known as trigeminal autonomic cephalalgias (TACs), are characterised by severe, strictly unilateral pain in the territory of the distribution of the trigeminal nerve, associated with autonomic manifestations. The recent International Headache Society classification lists the strictly unilateral headaches as cluster headache (CH), episodic and chronic paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. CH is the most common and best-defined of the TACs, whose pathophysiologies have not been adequately defined. Convincing proposals for pathophysiologic mechanisms must explain the trigeminal distribution of the pain, the homolateral autonomic manifestations; and, for CH, the usually periodic recurrence of the crises and clusters. With regard to CH, (i) the pain is always located periorbitally-frontally, implicating nociceptive mechanisms involving the trigeminal nerve; (ii) the autonomic manifestations homolateral to the pain seem to be both parasympathetic (lacrimation and rhinorrhoea) and sympathetic (ptosis and miosis); and (iii) the periodicity of the attacks and seasonal recurrence of the cluster periods suggest involvement of a biological clock within hypothalamus.

Autonomic Nervous System Diseases↗

Clomiphene citrate as a new treatment for SUNCT: hormonal manipulation for hypothalamic-influenced trigeminal autonomic cephalalgias.

We present a patient with treatment refractory short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) who was found to have low levels of serum testosterone supporting the hypothalamic connection to this trigeminal autonomic cephalalgia. Clomiphene citrate therapy induced a significant elevation of testosterone levels (by its effect on hypothalamic estrogen receptors) and led to a dramatic reduction in SUNCT attacks. Hormonal manipulation may be a treatment strategy for hypothalamic-influenced trigeminal autonomic cephalalgias.

Autonomic Nervous System Diseases↗

[Cluster headache and other trigeminal autonomic cephalalgias: diagnosis and treatment].

Cluster headaches are characterized by strictly unilateral paroxysmal attacks of severe pain with associated autonomic sign and symptom. Prevalence is 5 times higher in men than in women in our cases. About 10-15% of patients have chronic symptoms without remissions, but we estimated less frequent in Japanese (6.6% in our series). Pain almost invariably recurs on the same side, but in some patients (16.4%) the affected site switches. Cluster headache may be inherited in about 5% of our cases. Attacks frequently occur at night (60.7%). The patients (64.8%) are restless or agitated during an attack. Recent PET studies elucidated that acute attacks causes activation of the posterior hypothalamic grey matter. The excitement of the area might be responsible for peculiar clinical characteristics of agitation. Some patients (66.0%) have also have symptoms (especially a visual aura) usually attributed to migraine. Treatment of cluster headache includes both acute therapy aimed at aborting individual attacks and prophylactic therapy aimed at preventing recurrent attacks during the cluster period. There are many choices using for both therapies. Based on our clinical experience, we recommended the combination of nasal sumatriptan for acute attacks and verapamil 240 mg/day for prophylaxis.

Adolescent↗

Classification of primary headaches.

Given the range of disorders that produce headache, a systematic approach to classification and diagnosis is an essential prelude to clinical management. For the last 15 years, the diagnostic criteria of the International Headache Society (IHS) have been the accepted standard. The second edition of The International Classification of Headache Disorders (January 2004) reflects our improved understanding of some disorders and the identification of new disorders. Neurologists who treat headache should become familiar with the revised criteria. Like its predecessor, the second edition of the IHS classification separates headache into primary and secondary disorders. The four categories of primary headaches include migraine, tension-type headache, cluster headache and other trigeminal autonomic cephalalgias, and other primary headaches. There are eight categories of secondary headache. Important changes in the second edition include a restructuring of these criteria for migraine, a new subclassification of tension-type headache, introduction of the concept of trigeminal autonomic cephalalgias, and addition of previously unclassified primary headaches. Several disorders were eliminated or reclassified. In this article, the authors present an overview of the revised IHS classification, highlighting the primary headache disorders and their diagnostic criteria. They conclude by presenting an approach to headache diagnosis based upon these criteria.

Adult↗

Applying the International Classification of Headache Disorders to the emergency department: an assessment of reproducibility and the frequency with which a unique diagnosis can be assigned to every acute headache presentation.

STUDY OBJECTIVE: Although almost 2 decades have passed since the International Headache Society first introduced its International Classification of Headache Disorders (ICHD), the applicability of this classification scheme in the emergency department (ED) has not been assessed. As a first step toward identifying the role the ICHD should play in the ED, we address 2 questions: Can a structured interview and adherence to the ICHD allow ED headache patients to be classified in a reproducible manner? With the ICHD, how often can one specific diagnosis be assigned to each ED headache presentation? METHODS: This was a structured interview and medical record review of patients with nontraumatic headache, performed in an urban ED from March 2004 through August 2005. Using the data from the interview and the subject's ED record, 2 emergency medicine investigators independently classified each of the headaches twice: first, to determine presence or absence of a primary headache disorder, and then to determine presence or absence of a secondary headache disorder. If a primary headache was present, it was further classified as migraine, tension-type headache, trigeminal autonomic cephalalgia, chronic daily headache, or primary headache unclassifiable. Interobserver discordance was adjudicated by an experienced headache specialist. RESULTS: Four hundred eighty patients were enrolled in the study. The emergency medicine investigators had a high level of interobserver agreement on secondary headaches (agreement 94% [95% confidence interval (CI) 92% to 96%]) and primary headaches (agreement 91% [95% CI 88% to 93%]). Among the 480 subjects, 122 (25%) had a secondary headache disorder, 309 (64%) had a primary headache disorder, 49 (10%) had a coexisting primary and secondary headache, and for 95 (20%) subjects, neither a primary nor a secondary headache could be diagnosed. Of 309 subjects with a primary headache, 186 (60%) had migraine, 34 (11%) had tension-type headache, 2 (1%) had trigeminal autonomic cephalalgia, and 77 (26%) had an unclassifiable primary headache. Overall, a specific ICHD headache diagnosis could not be assigned to 36% of subjects either because a specific primary headache disorder could not be identified or because neither a primary nor a secondary headache disorder could be diagnosed. CONCLUSION: Although a detailed structured interview in the ED and adherence to the ICHD resulted in reproducible classification of headache patients, more than one third of acute headache patients could not readily be given a specific ICHD diagnosis in the ED.

Adult↗

Dental presentations of cluster headaches.

Cluster headache has been defined by the International Headache Society (IHS) as one of the primary headaches. A primary headache is a headache that has no other known cause, such as infection or trauma. Cluster headache is also listed as one of the trigeminal autonomic cephalalgias. These headaches are mediated by the trigeminal nerve with accompanying autonomic symptoms that may range from conjunctival injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, miosis, and ptosis to eyelid edema. The IHS has described cluster headache as "attacks of severe, strictly unilateral pain that is orbital, supraorbital, temporal or in any combination of these sites, lasting 15 to 180 minutes." In the author's practice, as a dentist treating orofacial pain, patients with cluster headache have dental or midfacial complaints as a primary presentation. This paper introduces such presentations based on interviews with cluster headache patients, with the main purpose of having midfacial complaints considered as an important presentation to be added to the IHS diagnostic criteria for cluster headache.

Adult↗

Autonomic headache with autonomic seizures: a case report.

The aim of the report is to present a case of an autonomic headache associated with autonomic seizures. A 19-year-old male who had had complex partial seizures for 15 years was admitted with autonomic complaints and left hemicranial headache, independent from seizures, that he had had for 2 years and were provoked by watching television. Brain magnetic resonance imaging showed right hippocampal sclerosis and electroencephalography revealed epileptic activity in right hemispheric areas. Treatment with valproic acid decreased the complaints. The headache did not fulfil the criteria for the diagnosis of trigeminal autonomic cephalalgias, and was different from epileptic headache, which was defined as a pressing type pain felt over the forehead for several minutes to a few hours. Although epileptic headache responds to anti-epileptics and the complaints of the present case decreased with antiepileptics, it has been suggested that the headache could be a non-trigeminal autonomic headache instead of an epileptic headache.

Anticonvulsants↗

SUNCT syndrome: an update.

SUNCT (Shortlasting Unilateral Neuralgiform Headache attacks with Conjunctival injection and Tearing) is a syndrome characterised by shortlived (5-240 s), strictly unilateral, orbital/periorbital, moderate-to-severe pain attacks, accompanied by rapidly developing conjunctival injection and lacrimation. Most attacks are triggered by mechanical stimuli, but there are also spontaneous attacks. Symptomatic periods alternate with remissions in an unpredictable fashion. In active periods, the attacks predominate during daytime, with a frequency that ranges from < 1 attack/day to > 30 attacks/h SUNCT is mainly a primary disorder, but is sometimes associated with intracranial structural lesions (symptomatic SUNCT). SUNCT has been included in the group of trigeminal autonomic cephalalgias, which are thought to depend on the activation of the trigeminal system together with the disinhibition of a trigeminofacial autonomic reflex. According to a few reports, SUNCT patients may benefit from carbamazepine, lamotrigine, gabapentin, topiramate or various surgical procedures. However, well-designed clinical trials are required before these therapeutic options can be sufficiently validated.

Carbamazepine↗

Rare primary headaches: clinical insights.

So-called "rare" headaches, whose prevalence rate is lower than 1% or is not known at all and have been reported in only a few dozen cases to date, constitute a very heterogeneous group. Those that are best characterised from the clinical point of view can be classified into forms with prominent autonomic features and forms with sparse or no autonomic features. Among the former are trigeminal autonomic cephalalgias (TACs) and hemicrania continua, while the latter comprise classical trigeminal neuralgia, hypnic headache, primary thunderclap headache, and exploding head syndrome. The major clinical discriminating factor for the differential diagnosis of TACs is the relationship between duration and frequency of attacks: the forms in which pain is shorter lived are those with the higher frequency of daily attacks. Other aspects to be considered are the time pattern of symptoms, intensity and timing of attacks, the patient's behaviour during the attacks, the presence of any triggering factors and of the refractory period after an induced attack, and response to therapy, especially with indomethacin. Often these are little known clinical entities, which are not easily detected in clinical practice. For some of them, e. g., thunderclap headache, it is always necessary to perform instrumental tests to exclude the presence of underlying organic diseases.

Diagnosis, Differential↗