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Cluster Headache associates a severe pain generally unilateral and autonomic symptoms with a remarkable periodicity. In the first part we tried to explain the conception of physiopathology of these short lasting headache syndromes and in the second part we described the clinical features. The short lasting primary headaches are divided into two groups: those with marked autonomic activation which comprise chronic and episodic paroxysmal hemicrania, short lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT syndrome) and cluster headache. The second group includes two entities, one without autonomic activation: Hypnic Headache and one with mild autonomic features: Hemicrania Continua. The paroxysmal hemicranias are characterized by attack frequency ranges from 15 to 20 per day of short lasting attacks of unilateral pain that typically last 2 to 10 minutes, the severe pain is associated with autonomic symptoms and responds to treatment with indomethacin. The SUNCT syndrome has a less severe pain but marked autonomic activation during attacks, this syndrome is actually resistant to proposed therapy. The Hypnic Headache and the Hemicrania Continua have yet less severe pain with very mild or without autonomic features.
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PMID:[Idiopathic facial pain other than vascular pain]. 1113 50

Cluster headache is a primary headache with well-defined diagnostic criteria. Nevertheless, in 3-5% of patients this syndrome is secondary to diverse cranial structural abnormalities. Atypical features which suggest a secondary or symptomatic origin include absence of periodicity and regular hourly recurrence, persistence of background pain among attacks, unsatisfactory response to treatment, and presence of neurological signs other than ptosis or miosis. We present two patients with symptomatic cluster-like headache. In one, the syndrome was associated with a fistula of the right superficial temporal artery. After embolization the pain attacks ceased. The second patient presented a ventricular xanthoma, located in the occipital horn; after surgical excision, the pain attacks did not recur. The progressive increase in frequency and absence of regularly hourly recurrence of pain episodes was a common feature in both cases. We comment and revise the pathophysiology of this syndrome, with emphasis on the case of the xanthoma, since we did not find a similar case described. If a secondary cluster-like headache is suspected, neuroimaging studies should be done.
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PMID:[Symptomatic cluster headache: presentation of 2 cases]. 1119 50

Cluster headache is one of the most excruciating headaches affecting human beings--especially the male sex. Most of the cluster headache cases are of episodic nature, with active cluster periods lasting generally between a few weeks and 2 or 3 months. A still undetermined percentage of patients report nonpainful sensations preceding the onset of the pain attack for a variable period of time. If occurring only a few minutes or a few hours before the onset of pain, such symptoms are called prodromal. When occurring for several days, weeks, or months before the pain, they are termed premonitory symptoms. The author believes that premonitory symptoms have not been properly diagnosed and emphasizes the need to investigate their presence, because by knowing them advances can be made in the understanding of the physiopathology of this particular cephalalgia. Furthermore, it can also allow the physician to be ahead, by giving preventive treatment and stopping or diminishing the intensity and duration of the pain attacks.
Curr Pain Headache Rep 2001 Feb
PMID:Premonitory symptoms in cluster headache. 1125 38

Cluster headache is an episodic form of primary neurovascular headache that is both severe and relatively rare. It is characterized by episodes of headache with cranial parasympathetic activation and sympathetic impairment that come in bouts, or clusters. Its pathophysiology can be divided into understanding the attack phenotype and the biotype of the periodicity. Acute attacks of cluster headache are marked by trigeminal nerve-mediated pain and with cranial autonomic activation, trigeminal-autonomic cephalalgia; an activation that characterizes the phenotype of a group of headaches. The signature feature of cluster headache is its periodicity, the daily cycle of attacks when the patient is in an active bout, or the circumannual, or other period, cycling that distinguishes the on period from the off period. Functional brain imaging with positron emission tomography and structural imaging with voxel-based morphometry have identified an area in the posterior hypothalamic gray as key in understanding cluster headache. This area is subtly enlarged in its gray matter volume, active during an acute cluster headache but inactive when patients are challenged between bouts. Cluster headache is likely to be a form of primary neurovascular pain whose phenotypic expression relies on the trigeminal-autonomic reflex, with a biotype determined by the brain area, the posterior hypothalamus, in which the lesion seems to be located. Understanding both the phenotypic expression and the biotype will, respectively, enable better acute attack treatments and better preventative management of this horrible form of headache.
Curr Pain Headache Rep 2001 Feb
PMID:Hypothalamic involvement and activation in cluster headache. 1125 39

Cluster headache is a rare, clinically well-characterized disabling disorder that occurs in both episodic and chronic forms. The very painful short-lived unilateral headache attacks are associated with autonomic dysfunction. A large number of drugs such as ergotamines, steroids, methysergide, lithium carbonate, verapamil, valproate, capsaicin, leuprolide, clonidine, methylergovine maleate, methylphenidate, and melatonin are considered beneficial for prophylaxis. Nevertheless, this extremely painful condition is occasionally refractory to conventional treatment. The antispastic agent baclofen has been shown to possess an antinociceptive activity. Its efficacy in neuralgias, central pain following spinal lesions, painful strokes, migraine, and medication misuse chronic daily headache suggests that it may be useful for prevention of cluster headache attacks. Therefore, we treated 16 symptomatic patients with cluster headache with daily baclofen, 15 to 30 mg, in three divided doses for the cluster period and 2 weeks after. Within a week, 12 patients reported the cessation of attacks. One was substantially better and became attack free by the end of the following week. In the remaining three patients, the attacks worsened and corticosteroids were prescribed. One of these was also given verapamil. Three of the 16 patients had an additional cluster period, which cleared with a second course of baclofen. In this pilot study, baclofen seemed to be effective, safe, and well tolerated for cluster headache, and seemed to retain its efficacy on repeated clusters.
Curr Pain Headache Rep 2001 Feb
PMID:The use of baclofen in cluster headache. 1125 42

Cluster headache is an uncommon yet well-defined neurovascular syndrome occurring in both episodic and chronic varieties. The most striking feature of cluster headache is the unmistakable circadian and circannual periodicity. Inheritance may play a role in some families. The attacks are of extreme intensity, of short duration, occur unilaterally, and are accompanied by signs and symptoms of autonomic dysfunction. In contrast to migraine, during an attack the cluster patient prefers to pace about. Attacks frequently occur at night. Although the pathophysiology of cluster headache remains to be fully elucidated, several seminal observations have recently been made. The medical 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. Agents used for acute therapy include inhalation of oxygen, sumatriptan, and dihydroergotamine. Transitional prophylaxis involves the short-term use of either corticosteroids or ergotamine derivatives. The cornerstone of maintenance prophylaxis is verapamil, yet methysergide, lithium, and divalproex sodium may also be employed. In some patients, melatonin or topiramate may be useful adjunctive therapies.
Curr Pain Headache Rep 2001 Feb
PMID:Treatment and management of cluster headache. 1125 43

Cluster headaches can be mimicked by a spontaneous carotid artery dissection. We report a 45-year-old man with a spontaneous carotid artery dissection whose unilateral headache responded to sumatriptan. An oral dose of 50 mg of sumatriptan relieved 90% of the pain after 2 hours. A second dose the next day achieved similar results within 4 hours. The diagnosis of dissection was made later by magnetic resonance angiogram and conventional angiography. This case illustrates that a positive response to a triptan can not be used to distinguish the first attack of cluster headache from a carotid artery dissection.
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PMID:Sumatriptan can alleviate headaches due to carotid artery dissection. 1143 96

Cluster headache is usually considered to affect young men. We hereby report on new-onset cluster headache in middle-aged and elderly women. We performed a retrospective chart review of female patients diagnosed with cluster headache (IHS criteria), and studied the charts of women in whom the headache started after the age of 50 years. Out of 168 patients (26 women, 15%) diagnosed with cluster headache, the headache started after the age of 50 years in seven women, of whom three reported past tension-type or migraine headaches. The mean age at the beginning of the headache was 61 +/- 8 years (range 52--72 years). In all cases, the pain was severe, strictly unilateral, and accompanied by at least one autonomic symptom. The average duration of the pain was 70 min (range 20 min--3 h), recurring daily for an average period of 7 weeks (range 1--16 weeks). Five patients had 1--2 pain attacks each day, while the other two experienced up to eight episodes of pain in 1 day. In two patients the periodicity of the pain was currently undetermined. In the remainder, the headache periods recurred every 1--4 years. Cluster headache is commonly considered to be a young-male disorder, but middle-aged and elderly women may also be affected. The characteristics of the pain and its manner of occurrence were similar in our cases to those reported in the young-male population.
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PMID:New-onset cluster headache in middle-age and elderly women. 1144 54

Cognitive-behavioral analysis and the multiaxial assessment of relevant behavioral domains (headache frequency and severity, analgesic and abortive use and misuse, behavioral and stress-related risk factors, comorbid psychiatric disorders, and degree of overall functional impairment) help set the stage for CBT of headache disorders. Controlled studies of CBTs for migraine, such as biofeedback and relaxation therapy, have a prophylactic efficacy of about 50%, roughly equivalent to propranolol. Cluster headache responds poorly to behavioral treatment. The persistent overuse of symptomatic medication impedes the effectiveness of behavioral and prophylactic medical therapies. Behavioral treatment can help sustain improvement after analgesic withdrawal, however, and prevent relapse in cases of analgesic overuse. Cognitive factors (e.g., an enhanced sense of self-efficacy and internal locus of control) appear to be important mediators of successful behavioral treatment. Patients with CDH are more likely to overuse symptomatic medication (and in some cases abuse analgesics), have more psychiatric comorbidity; have more functional impairment and disability, and are at least as likely to experience stress-related intensification of headache as patients whose episodic headaches occur less than 15 days per month. Despite the significance of these behavioral factors, patients with CDH (particularly those with migrainous features) are less likely to benefit from behavioral treatment without concomitant prophylactic medication than is the case for episodic TTH and migraine sufferers. Continuous daily pain may be more refractory to behavioral treatment as a solo modality than CDH marked by at least some pain-free days or periods of time. The combination of behavioral therapies with prophylactic medication creates a synergistic effect, increasing efficacy beyond either type of treatment alone. Compliance-enhancement techniques, including behavioral contracts for patients with severe personality disorders, can increase adherence to behavioral recommendations. CBT has earned an important place in the comprehensive treatment of patients with episodic migraine/TTH and severe, treatment-resistant chronic daily headache.
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PMID:Behavioral and nonpharmacologic treatments of headache. 1148 Feb 58

The disorders described in this article are relatively rare, but probably are more common than previously thought. Because these disorders cause significant pain and disability and treatment response differs from that of migraine, tension-type, and cluster headaches, recognition is essential. Table 1 lists the important clinical features of these syndromes and contrasts them with cluster headache, the disorder for which they are often confused.
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PMID:Cluster and related headaches. 1148 Feb 70


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