Behavior during cluster headache

Cluster headache (CH), also known as “suicide headache,” is characterized by a distinctive behavior during attacks. In 80% to 90% of cases, patients are restless and constantly moving in a vain attempt to relieve pain. They often perform complex, stereotyped actions. During attacks, CH sufferers do not want to be touched, stroked, or comforted and frequently moan a great deal, cry, or even scream. They sometimes indulge in violent, self-hurting behavior. Restlessness is a highly sensitive and highly specific parameter for CH and has been included among the signs and symptoms accompanying pain of the disorder in the Second Edition of the International Classification of Headache Disorders. A few hypotheses on pathophysiology of restlessness are addressed in this paper.     

Cluster Belly: A Variant of Irritable Bowel Syndrome

Cluster headache (CH) and irritable bowel syndrome (IBS) are pain disorders that possess relationships with circadian rhythms. However, they have not been compared to assess similarities that could yield pathophysiologic insights. A young male adult with periodic episodes of abdominal pain highly reminiscent of CH is described. Since childhood, he experienced severe attacks featuring excruciating, abdominal pain accompanied by prominent restlessness, lasting 30‐120 minutes, occurring in the evening and in discrete 2‐ to 8‐week periods, interspersed with remissions where typical triggers did not lead to attacks. Although all of the patient's symptoms fell within the spectrum of IBS, the semiology was highly evocative of CH, based on the attack duration, restlessness, periodicity, and selective vulnerability to particular triggers only during attack periods. A subset of patients thought to have IBS may feature similar attack profiles and could suggest the importance of the hypothalamus in its pathophysiology, akin to CH.     

Application of the International Headache Society classification criteria in 652 cluster headache patients

We applied the International Headache Society (IHS) classification coding parameters to a study population of 652 cluster headache (CH) patients, in order to determine how many patients did not fulfil the diagnostic criteria for group 3.1 and to find out any diagnostic elements that could be changed in the upcoming revision of the classification to make it more relevant to current clinical practice. Ninety-nine patients were found to have cluster-like disorder (3.3), including 74 (74.7%) who did not fulfil the diagnostic criteria for CH, because either pain was not associated with any of the accompanying autonomic phenomena listed in the classification or it was not located orbitally, supraorbitally and/or temporally. A review of our total sample showed that 72.0% of patients reported frontal and occipital pain location; in 61.8%, 33.4% and 39.1% of cases, attacks were also accompanied by restlessness/agitation, nausea and photophobia, respectively. In a coding system that took into account the diagnostic elements that we considered in our study, group 3.1 of the existing IHS classification would actually include 51 of the 99 patients currently coded as 3.3.     

The Primary Headaches as a Reflection of Genetic Darwinian Adaptive Behavioral Responses

(Headache 2010;50:273‐289) Objective.— The objective of this study is to present a view of the primary headaches as genetically determined behavioral responses consistent with sickness behavior and defense reaction, respectively. Background and Design.— A review of the literature bearing on the behavioral, humoral, and functional imaging aspects of the primary headaches shows that migraine and cluster headache (CH) are pain conditions characterized by different behaviors during the attacks. Here it is postulated that the behavioral responses to migraine and CH are evolutionary conserved reactions consistent with sickness behavior and defense reaction. Results.— The sickness behavior observed during migraine attacks is a pan‐mammalian adaptive response to internal and external stressors, characterized by withdrawal and motor quiescence, sympatho‐inhibition and lethargy, in which visceral pain signals a homeostatic imbalance of the body and/or brain. In contrast, the defense reaction in CH consists of a fight‐or‐flight reaction, with motor restlessness and agitation, in which pain is exteroceptive in kind. Conclusion.— These different behavioral responses are thus specific to different kinds of pain, distinguished by the behavioral significance of the pain (visceral pain in migraine vs exteroceptive pain in CH), and imply brain matrices involving different networks in the brainstem, hypothalamus, and forebrain regions that engender evolutionarily conserved adaptive genetic responses. Cytokines play an important role in their development. Predictions and limitations of the hypothesis are discussed together with implications for genetic studies on headaches.     

Assymetry of Temporal Artery Diameters During Spontaneous Attacks of Cluster Headache

Background.— Cluster headache is characterized by strictly unilateral head pain associated with symptoms of cranial autonomic features. Transcranial Doppler studies showed in most studies a bilateral decreased blood flow velocity in the middle cerebral artery.
Objective.— To investigate whether there is a bilateral or unilateral extracranial vasodilation during spontaneous cluster headache attacks.
Design and methods.— In 9 cluster headache patients, we investigated the luminal diameter of the superficial temporal artery with ultrasound on the headache and headache-free side during and outside cluster headache attacks.
Results.— During cluster headache attacks, the diameter of the superficial temporal artery on the painful side was greater, 1.48 mm, than the diameter on the nonheadache site, 1.14 mm ( P  < .01). Outside attacks, median diameters on the 2 sides were quite comparable, 1.34 vs 1.31 mm ( P  = .67).
Conclusions.— What was observed is most likely a general pain-induced arterial vasoconstriction (confer the decrease in diameter on the pain-free side) with an unchanged superficial temporal artery on the pain side because of some vasodilator influence.     

Accompanying symptoms of cluster attacks: their relevance to the diagnostic criteria

Two-hundred-and-fifty-one consecutive cluster headache (CH) patients referred to the Pavia and Parma Headache Centers were evaluated in order to verify the presence and recurrence of one or more autonomic symptoms. Data obtained show that in 2.8% of patients cluster attacks were not accompanied by localized autonomic symptoms, thus confirming the report of Ekbom. We observed a high prevalence of photophobia, nausea and vomiting. The IHS diagnostic criteria for CH may need to be modified. The high frequency of "general" autonomic symptoms seems to suggest a component of "central" drive in the physiopathology of cluster headache.     

"Mild" cluster headache(?)

Three cases of what could be considered "mild" cluster headache have been described. All three patients were generally able to carry out their work during attacks; all three were men and had unilateral headache with the predominant pain in the ocular region. Relatively few symptoms and signs indicated autonomic system involvement, but at least tearing was invariably present on the symptomatic side. The bouts were generally short-lasting in two of the patients (and partly in the third one), fitting the pattern of "mini-bouts". Thus, in one of the cases four of the five major criteria (male sex, excruciating severity, cluster phenomenon, autonomic involvement, and unilaterality) were present. In the two other patients the full-blown cluster phenomenon was lacking. Such cases may represent the left-side slope of a "Gaussian severity distribution scale" with regard to cluster headache.     

Validation of a questionnaire for the detection of cluster headache

BACKGROUND: In the absence of biological markers, the diagnosis of cluster headache (CH) rests on clinical evidence as reported through ad-hoc interviews. OBJECTIVE: The aim of this study was to validate a 16-item self-administered questionnaire designed to screen CH cases. METHODS: The questions were based on the second edition of the International Headache Society classification (ICHD-II) criteria for CH (n = 12) and on specific disease features (n = 3). Answers to each question were either "Yes,"No," or "Don't know." The validity of this screening tool was assessed using a two-step procedure. In Step 1, the 16 questions were submitted to 30 healthy subjects with different cultural backgrounds to verify content clarity. In Step 2, the questionnaire was given to 71 patients (32 women and 39 men) aged 15 to 78 years (mean 37.5 years), who were seen at the University of Parma Headache Center with a diagnosis of CH (episodic, 17; chronic, 13), migraine (without aura, 18; with aura, 3), and tension-type headache (episodic, 16; chronic, 4) according to the ICHD-II criteria. Sensitivity, specificity, and the positive and negative predictive values were calculated for each question as measures of validity. RESULTS: Severity, unilaterality, and location of pain had the highest sensitivity (100%), but low (34.1%) or fairly low specificity (61.0% and 58.5%, respectively). In contrast, a positive response to lithium or verapamil had a 66.7% sensitivity and a 97.6% specificity. Sensitivity and specificity were 100% and 90.2% for duration of attacks (<180 to 240 minutes), and 90.0% and 92.7% for compulsory movements, respectively. The best discriminatory pattern for symptom detection was unilaterality of pain and the presence of at least five of the following seven features: pain severity and location, duration, frequency and daily recurrence of attacks, rhinorrhea and restlessness as accompanying symptoms. CONCLUSIONS: Our findings confirm that this questionnaire is a useful method for CH screening in epidemiological studies.     

Cluster headache--acute and prophylactic therapy

Cluster headache (CH) pain is the most severe of the primary headache syndromes. It is characterized by periodic attacks of strictly unilateral pain associated with ipsilateral cranial autonomic symptoms. The majority of patients have episodic CH, with cluster periods that typically occur in a circannual rhythm, while 10% suffer from the chronic form, with no significant remissions between cluster periods. Sumatriptan injection or oxygen inhalation is the first-line therapy for acute CH attacks, with the majority of patients responding to either treatment. The calcium channel blocker verapamil is the drug of choice for CH prevention. Other drugs that may be used for this purpose include lithium carbonate, topiramate, valproic acid, gabapentin, and baclofen. Transitional prophylaxis, most commonly using corticosteroids, helps to control the attacks at the beginning of a cluster period. Peripheral neural blockade is effective for short-term pain control. Recently, the therapeutic options for refractory CH patients have expanded with the emergence of both peripheral (mostly occipital nerve) and central (hypothalamic) neurostimulation. With the emergence of these novel treatments, the role of ablative surgery in CH has declined.     

Validation of a Brief Self-Administered Questionnaire for Cluster Headache Screening in a Tertiary Center

Background.— Cluster headache remains substantially underdiagnosed and undertreated. Early neurologic referral is indicated in patients with a suspected diagnosis of cluster headache (CH) so that management can be optimized and unnecessary procedures avoided.
Objective.— To validate a brief self-administered questionnaire designed to screen CH cases in tertiary centers.
Methods.— The review of clinical studies led us to identify the 3 more prevalent criteria of the second edition of the International Headache Society classification (International Classification of Headache Disorders, 2nd edition [ICHD II]) for all forms of CH (episodic and chronic forms). These 3 criteria were: strictly unilaterality of pain, attack duration <180 minutes if untreated, ipsilateral conjunctival injection, and/or lacrimation. These criteria were transformed in questions formulated in such a way that they could be self-administered and easily understood. Answer to each question was yes or no. Patients were unaided. The self-questionnaire was compared with the gold standard, the ICHD II criteria used by specialists at the university of Bordeaux headache center. We calculated the sensitivity and specificity for the 3 questions and for each pair of questions.
Results.— The self-questionnaire was consecutively and prospectively submitted to 37 patients with CH and 59 patients with migraine. The 3-item questionnaire had a 78.4% sensibility and a 100% specificity. The 2-item questionnaire only using the attack duration associated with conjunctival injection and/or lacrimation was more sensitive (81.1%) with the same specificity (100%).
Conclusions.— This 2-item questionnaire could be a useful tool for screening CH cases in tertiary centers.     

Baclofen in cluster headache

Cluster headache is a rare, severe, clinically well-characterized disorder that occurs in both episodic and chronic forms. The painful short-lived attacks occur unilaterally and are associated with signs and symptoms of autonomic involvement. They are difficult to treat, and reported prophylactic therapies include ergotamine, steroids, methysergide, lithium carbonate, verapamil, valproate, capsaicin, leuprolide, clonidine, methylergonovine maleate, and melatonin. Baclofen, an antispastic agent, has been shown to have an antinociceptive action. Its efficacy in the treatment of neuralgias, central pain following spinal lesions or painful strokes, migraine, and medication misuse chronic daily headache suggested that it may prevent cluster headache attacks. Nine cluster headache patients received baclofen, 15 to 30 mg, in three divided doses. Within a week, six of nine patients reported the cessation of attacks. One was substantially better and became attack free by the end of the following week. In the remaining two patients, the attacks worsened and corticosteroids were prescribed. In this pilot study, baclofen seemed to be effective and well tolerated for the prevention of cluster headache.     

Cluster Headache as the Index Event in MS: A Case Report

We report a 42‐year‐old woman who presented with cluster headache (CH) in association with other neurological symptoms as the index event of new onset multiple sclerosis (MS). Her initial symptoms were left‐sided headache with ipsilateral lacrimation and nasal congestion associated with ipsilateral facial numbness. A subsequent similar headache attack was also associated with ipsilateral arm ataxia and gait ataxia. She had many additional short headache attacks without focal neurological symptoms. Her cluster‐like headache attacks have not recurred since intiation of dimethyl fumarate. Our patient illustrates that cluster‐like headache attacks can occur as a first symptom of MS, in our patient in association with other neurological symptoms. A striking finding in our patient was a large demyelinating lesion in the brachium pontis ipsilateral to the headaches, although additional supratentorial demyelinating lesions were also present. Although CH associated with MS is rare, our patient and the two other reported patients with MS and CH with similar ipsilateral brachium pontis lesions suggest that the lesions in this location may have played a role in the generation of the cluster‐like attacks.     

Extratrigeminal Cluster Headache

SYNOPSIS
Three cases with periodicity and pain profile characteristic of episodic cluster headache, whose headaches were solely confined to the regions of the head and neck outside the trigeminal territory, are reported. Two were females, who had associated nausea and vomiting with severe attacks. The male patient exhibited autonomic symptoms in the eye during the attacks. Alcohol induced headache in one. All three patients responded to anticluster headache therapy.
These cases are illustrative of a wider spectrum of clinical manifestations of cluster headache than was originally recognized. They question the theory that cluster headache may be due to a lesion involving the cavernous sinus. One the other hand, it points to involvement of a more complex pain circuit consisting of upper cervical nerves, posterior fossa innervation, trigeminal system and the autonomic pathways.     

"Cervicogenic headache": Clinical manifestation

The main criteria of "cervicogenic headache" are considered to be as follows: relatively rare and long-lasting unilateral attacks of severe headache, although seemingly of a non-excruciating character, signs of neck involvement, and lack of "cluster pattern". In the present communication, the clinical manifestations in 11 patients fulfilling these criteria are described. All 11 patients selected in accordance with these criteria proved to be females, the age at onset ranging from 6 to 40 years (mean, 30 years). The mean duration of symptoms was 13 years. Six patients had had previous head/neck injuries. All patients had pain periorbitally, in the temporal region, and in the low occipital region (nape of the neck); less frequent were frontal, parietal, and facial pain and pain in the upper part of the occipital region. The duration of attacks was from 3 h to 3 weeks, and the interval between attacks lasted from 2 days to 2 months. The commonest accompanying phenomena were phonophobia, dizziness, ipsilateral eyelid edema, ipsilaterally blurred vision, and irritability. Some of the patients also had nausea (n = 7) and vomiting (n = 6). On physical examination, slight to moderate reduction of movements in the neck was noted, and five patients had ipsilaterally reduced sensation for touch in the trigeminal area. All the patients except one were severely afflicted. Attacks could, in addition to occurring spontaneously, be precipitated in all patients by head movements or by pressure at specific points in the neck.     

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.     

Peripheral postganglionic sympathicoplegia mimicking cluster headache attacks

After dissection with complete occlusion of the internal carotid artery, a 58-year-old man started to suffer from intense cluster headache-like attacks. Magnetic imaging showed signs of nonsymptomatic cerebral emboli, which could be dated to have occurred in temporal relation to the start of the attacks, all on the right side. This case and two similar ones indicate that peripheral postganglionic sympathicoplegia can cause attacks with similar pain characteristics, accompanying symptoms, duration, and regularity as in cluster headache.     

Subcutaneous sumatriptan induces changes in frequency pattern in cluster headache patients

OBJECTIVES: To document the relationship between the use of subcutaneous (SQ) sumatriptan (sum) and a change in frequency pattern of cluster headache (CH) in six patients. To discuss the clinical and pathophysiological implications of this observation in the context of available literature. BACKGROUND: Treatment with SQ sum may cause an increase in attack frequency of CH but data from literature are scant and controversial. METHODS: Six CH sum-na?ve patients (three episodic and three chronic according to the International Headache Society (IHS) criteria) are described. RESULTS: All six patients had very fast relief from pain and accompanying symptoms from the drug but they developed an increase in attack frequency soon after using SQ sum. In all patients, the CH returned to its usual frequency within a few days after SQ sum was withdrawn or replaced with other drugs. Five patients were not taking any prophylactic treatment and SQ sum was the only drug prescribed to treat their headache. CONCLUSIONS: Physicians should recognize the possibility that treatment of CH with SQ sum may be associated with an increased frequency of headache attacks.     

Response of Cluster Headache to Kudzu

Objective.— To describe the self-treatment of cluster headache with kudzu.
Background.— Many cluster headache patients take over-the-counter (OTC) kudzu extract in the belief that it helps their cluster attacks. Kudzu's actual efficacy has not been studied.
Methods.— A database of cluster headache patients was questioned about their use of various alternative remedies to treat their cluster headache. Of 235 patients identified, 16 had used kudzu, consented to interviews, and provided medical records.
Results.— In total, 11 (69%) experienced decreased intensity of attacks, 9 (56%) decreased frequency, and 5 (31%) decreased duration, with minimal side effects.
Conclusion.— Anecdotal evidence suggests that a component in OTC products labeled as kudzu may prove useful in managing cluster headache. This hypothesis should be tested with a randomized clinical trial.     

Evaluating the IHS criteria for cluster headache--a comparison between patients meeting all criteria and patients failing one criterion

Cluster headache (CH) is diagnosed according to criteria of the International Headache Society (IHS), but, in clinical practice, these criteria seem too restrictive. As part of a nation-wide study, we identified a group of patients who met all criteria minus one (IHS-CH-1), and assessed in which way they differed from CH patients meeting all criteria (IHS-CH). We performed a nation-wide questionnaire study for CH and CH-like syndromes, including questions based on the IHS criteria, and additional features such as restlessness during attacks, nocturnal onset of attacks, circadian rhythmicity of attacks and response to treatment. IHS-CH and IHS-CH-1 patients were compared. Of 1452 responders to two questionnaires, 1163 were IHS-CH and 289 were IHS-CH-1. The majority of the IHS-CH-1 patients were classified as such because their attacks exceeded 3 h (64%, median attack duration: 5 h), or came in a frequency of less than 1 per 2 days (16%). Age at onset was similar between the groups. The male to female ratio was 3.7 : 1 in the IHS-CH group and around 1.6 : 1 in the IHS-CH-1 groups (P < 0.005). Patients with attacks exceeding 3 h less often reported a circadian rhythmicity (IHS-CH-1: 49%, IHS-CH: 64%), episodic periodicity (IHS-CH-1: 65%, IHS-CH: 78%), nocturnal attacks (IHS-CH-1: 67%, IHS-CH: 78%), smoking (IHS-CH-1: 90%, IHS-CH: 80%) and restlessness during attacks (IHS-CH-1: 64%, IHS-CH: 76%) than IHS-CH patients (P < 0.005). Photo- or phono-phobia (IHS-CH-1: 67%, IHS-CH: 54%) and nausea (IHS-CH-1: 38%, IHS-CH: 27%) were more frequently reported by patients who reported to have attacks exceeding 3 h (P < 0.005). Similar proportions reported effect of verapamil on their attacks (IHS-CH-1: 54%, IHS-CH 61%). We conclude that average attack duration exceeding 3 h was frequently the reason for not fulfilling IHS CH criteria. Symptoms often accompanying CH such as restlessness, nocturnal attacks and an episodic attack pattern were relatively frequently present in IHS-CH-1 patients with longer attacks. These patients may therefore be diagnosed with CH. Attack frequency may not be a useful criterion for the diagnosis of CH. The upper limit of 3 h should be increased in future diagnostic criteria.