The sweating anomaly in cluster headache

We describe a patient with a typical history of cluster headache for more than 18 years. During the first approximately 10 years of his disease, the pain was right-sided, and pupillometric and evaporimetric measurements indicated a sympathetic deficiency on this same side. However, for the next >6 years, his pain was consistently left-sided, although the signs of sympathetic dysfunction still were more marked on the right side. This was also true for the findings obtained during the interictal period and for the heating test performed within an attack. The implications of this interesting case are discussed. The view that two separate lines of symptom production lead to the pain and the autonomic phenomena seems to be supported by this case history. The cluster headache syndrome may also be a bilateral disorder, with only the weight of balance pointing one way or the other. Finally, the autonomic test results of this patient could reflect an autonomic "scar" in the previous headache side.     

Cluster headache: On the significance of so-called minibouts

Cluster headache periods (bouts) have, at the lower end of the scale, been supposed to last for periods as short as 1-2 weeks. Patients who usually have bouts of ordinary duration occasionally have brief attacks in the remission phase ("abortive attacks"). The group described here had a temporal pattern, clearly at variance with both these patterns: The six patients had mostly or entirely bouts of less than 1 week's duration. We have termed such short-lasting bouts minibouts, on the supposition that such cases belong within the cluster headache cycle. All the patients were men and had a history of unilateral headache without side shift, and usually the solitary attacks had some autonomic accompaniments, although of a moderate degree. In four cases only mild attacks occurred, whereas in two cases the attacks were rather severe and not consistent with full working ability. The group as such may not be homogeneous. The nosologic status of patients with bouts lasting only 1-2 days is somewhat uncertain. As long as the substrate of cluster headache is not outlined, the lower end of duration of minibouts cannot be accurately identified. The fact that three patients recently have experienced bouts of ordinary length strengthens the view that such cases really belong to the cluster headache cycle.     

Burden of cluster headache

The aim was to analyse the socioeconomic burden of cluster headache in patients from a tertiary headache centre. One hundred consecutive patients from the Danish Headache Centre were invited to an interview about the socioeconomic impact of cluster headache. Work absence and use of medical services were compared with a Danish population-based survey. Eighty-five patients participated; 78% reported restrictions in daily living and 13% also outside of cluster periods; 25% reported a major decrease in their ability to participate in social activities, family life and housework. The disease caused lifestyle changes for 96%, most frequently in sleeping habits and avoidance of alcohol. The absence rate among patients was 30%, which was significantly higher than 12% among the general population (P < 0.001). Use of health services due to headache was also higher among the patients (P < 0.001). Cluster headache, although periodic in most cases, has considerable impact on social functions, quality of life and use of healthcare.     

Valsalva-induced cluster headache

A case valsalva-induced cluster headache is presented. Cluster attacks induced by valsalva manoeuvres alone were only recently described, and such patients have features of cough headache and cluster headache. Attacks occurred a couple of times a week in the patient presented, solely triggered by valsalva manoeuvres including coughing, sneezing or straining and not by exercising.     

Cluster headache: Forehead sweating pattern during heating and pilocarpine tests

Thirty-one patients with cluster headache were examined with regard to their forehead sweating pattern, by means of the Evaporimeter. Sweating was stimulated in two different ways: by body heating and by parenterally administered pilocarpine. The resulting increase in evaporation was frequently measured at different positions on both sides of the forehead, and the possibility of variations in the pattern related to the passage of time was specifically scrutinized. Some typical patterns emerged. The previously reported, marked asymmetries of response (deficient heat-induced sweating and pilocarpine supersensitivity of the symptomatic side) at the medial positions in the forehead were confirmed. However, the asymmetries invariably faded to some extent with the passage of time. Patients with cluster headache show gross similarities with, but also some minor differences from, the sweat pattern of patients with brain stem lesions causing a Horner's syndrome. A subdivision of the material into groups in accordance with the pupillometric pattern after sympathomimetic stimulation made it clear that the cases of definite evaporimetric asymmetries ("typical reactions") belonged to the group with a typical pupillometric pattern. These results suggest that from an "autonomic" point of view, subpopulations may exist within the clinical entity of cluster headache.     

Cluster headache: pupillometric patterns as a function of the degree of anisocoria

Fifty-three patients with cluster headache, mean age 42.6 years, were examined by means of pupillometry. Pharmacological stimulation was carried out by the instillation of eye drops; the sympathomimetic agents hydroxyamphetamine (a norepinephrine releaser) and phenylephrine (an agent acting directly on the postsynaptic receptors) were applied. Pupillary dilatation was measured at set time intervals, comparing the responses of the symptomatic and non-symptomatic sides. The material was divided into groups according to the degree of basal anisocoria. The subgroup with the most pronounced basal miosis of the symptomatic side demonstrated a uniform pattern of deficient symptomatic side dilatation after OH-amphetamine and supersensitivity to phenylephrine. The other groups demonstrated the same general pattern, but to a far lesser degree. In cluster headache, the extent of nonresponsiveness to OH-amphetamine and of phenylephrine supersensitivity on the symptomatic side thus, at least partly, seems to be a function of the degree of anisocoria. The response pattern in cluster headache seems to differ from that of 3rd neuron Horner's syndrome with an anisocoria of the same extent.     

Orbital phlebography in patients with cluster headache

Thirteen patients with cluster headache in an active stage were investigated with orbital phlebography. About 60% of the patients showed pathologic changes on the phlebograms, such as changes in the appearance of the superior ophthalmic vein. Five patients had pathologic changes on both sides and three patients on one side only. All patients with unilateral pathologic findings on orbital phlebography had the attacks on the same side. The phlebographic findings in these patients with cluster headache were very similar to those of patients with the Tolosa-Hunt syndrome. There is also some similarity in the symptoms in the two disorders. It has previously been suggested that the Tolosa-Hunt syndrome is caused by venous vasculitis, and the present findings to some extent support the idea that cluster headache may have the same etiology.     

Migraine in patients with cluster headache

One hundred and twenty seven patients with cluster headache have been compared with 122 patients with migraine. Twenty of the patients with cluster headache have had migraine attacks but only 2 still have migraine attacks after the onset of cluster headache. No migraine patients had cluster headache. Among the 127 patients with cluster headache, one of the parents suffered from cluster headache in 4.7% of the cases. Among the 122 patients with migraine, 0.8% had a parent with cluster headache. Among the 122 patients with migraine 54.9% had parents with migraine, and in the cluster headache group 23.6% of the patients had one parent with migraine. The coexistence of migraine and cluster headache is rare. The two types of headache, as far as the heredity pattern is concerned, are independent entities.     

A case of parasellar meningioma mimicking cluster headache

A medical history of a 46-year-old male is reported. At 23 years of age, he started having diffuse pain in the left side of his head for up to 30 min once or twice a month. At 28, the pain changed into left-sided cluster headache-like attacks with 2-3 h duration and with ipsilateral conjunctival injection, lacrimation, and rhinnorhea, but with short-lasting free intervals of about two to three weeks. At 36, the pattern of the attacks corresponded to chronic migrainous neuralgia. At 40, the symptoms changed to painful ophthalmoplegia-picture. A left-sided parasellar meningioma was then diagnosed. Removal of the tumor caused complete amelioration. The case history is suggested to support the hypothesis that the cavernous sinus region is involved in cluster headache.     

Hereditary haemochromatosis in two cousins with cluster headache

A 60-year-old woman with secondary chronic cluster headache had increased serum ferritin and serum transferrin saturation and was homozygous for the C282Y mutation in the HFE gene, which is indicative of hereditary haemochromatosis. Treatment with venesection that normalized her iron stores led to a radical improvement of her headache complaints that had been daily for several years. Later, the headache returned to some degree in spite of normal serum ferritin levels. Her cousin, a 33-year-old man who had had episodic cluster headache for several years, also had increased transferrin saturation and was compound heterozygous for two mutations, a genotype known to be associated with a slightly increased frequency of haemochromatosis. This is the first report of a headache disorder in a patient with hereditary haemochromatosis. The coexistence of the two disorders may be a mere coincidence, but the temporary improvement of headache from depletion of iron stores may indicate a causal relation, possibly mediated by iron deposits in pain-modulating centres in the brainstem.     

New-onset cluster headache in middle-age and elderly women

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.     

Premonitory and prodromal symptoms in cluster headache

Warning symptoms in 150 cluster headache patients were studied by focusing on attacks occurring during waking hours. Warnings were divided into prodromes that started minutes before the pain of individual attacks (122 patients) and premonitory symptoms preceding the onset of cluster periods by days to weeks (12 patients). Pathogenetic and therapeutic implications are discussed.     

Episodic cluster headache and narcolepsy: a case report

A patient who first presented with episodic cluster headache later developed narcolepsy. In spite of REM sleep alterations associated with narcolepsy, the frequency and distribution of pain attacks did not change when narcolepsy occurred and were similar to those seen in cases of episodic cluster without narcolepsy. The lack of influence of narcolepsy on the pattern of cluster pains questions the role of REM sleep states in triggering pain in episodic cluster.     

Acute pharmacotherapy of migraine,tension-type headache,and cluster headache

Abstract In most migraine patients acute therapy is needed. Migraine can be treated either with specific drugs, the triptans and ergot alkaloids, or with NSAIDs. Triptans are a major step foreward in migraine therapy. The therapeutic gain for headache relief is 50% for subcutaneous sumatriptan whereas it is 30-40% for most oral triptans. After oral triptans sustained pain free is only 30%. There is thus still ample room for improvement of acute therapy in migraine. For tension-type headache there is no specific therapy and it is treated with NSAIDs. Only 17-32% become pain free after these drugs. For attacks of cluster headache oxygen and subcutaneous sumatriptan can be used. Intranasal triptans can be an alternative.     

Prevalence of cluster headache in a population-based sample in Germany

A population-based sample of 6,000 inhabitants of the city of Essen in Germany was screened using a standard questionnaire for possible cluster headache (CH). Fifty-six percent responded (N = 3336, 50.5% of them women, mean age 44.7 +/- 12.7 years). All suspected cases (N = 182) were interviewed by a neurologist. Four subjects with CH (three men) were identified. The 1-year prevalence of CH was estimated to be 119/100,000 (95% confidence interval 3, 238/100,000).     

Management of cluster headache in the Emergency Department

Although cluster headache (CH) is considered one of the most distinctive and painful primary headache disorders in clinical practice because of the brevity of each attack, its management is not always ergonomic or possible in the Emergency Department. In case of a previously competent diagnosis, the Emergency Department’s team should send the patient to a headache centre where specialists in the management of CH can handle the pathology in the best way. In our headache centre we treat patients with CH attacks with a hyperbaric chamber, confirming the effectiveness of hyperbaric oxygen in CH patients.     

Effects of increasing the intracranial blood volume in cluster headache patients and controls

Eleven patients with episodic cluster headache in period, five patients out of period and six controls were studied concerning the effects of an increase of the intracranial blood volume by tilting. Common carotid artery (CCA) blood flow was similar in all three groups at baseline and during tilting. CCA diameters were similar at baseline and increased during tilting in all three groups, indicating that tilting caused an increase in the extra- and intracranial blood volume. Unilateral pain or sympathetic dysfunction did not appear during tilting in the patients out of period or in the controls. In four of eight studied patients with cluster headache in period, unilateral miosis and ptosis appeared during tilting. Two of these four patients developed intense unilateral pain, while the other two did not report any pain. Four other patients developed slight unilateral pain but no sympathetic dysfunction during tilting.     

Dihydroergotamine nasal spray in the treatment of attacks of cluster headache

A double-blind trial of dihydroergotamine (DHE) nasal spray compared with placebo was carried out in patients with cluster headache. Twenty-five patients were included in the trial. In three patients, all receiving DHE, the pain attacks ceased after five attacks. In the other 22 patients, 133 attacks were treated with placebo and 137 attacks with DHE nasal spray (dosage, 1 mg of DHE). The trial showed that the treatment given has no effect on the attack frequency or the duration of the single attack. However, the treatment had a significant effect on the intensity of the single attacks. It can be concluded that the trial should be repeated, using a larger dosage of DHE. This should be ethically justifiable, since none of the patients had any adverse reactions locally in the mucous membrane of the nose or systemically.     

Analysis of nitric oxide synthase genes in cluster headache

The aetiology of cluster headache is still not yet completely understood, but the potential relevance of genetic factors has been recognized during recent years. Nitric oxide (NO) plays a critical role in the regulation of vasodilation, neurotransmission, inflammation and many other events throughout the body. NO also appears to be an important mediator of vascular headache pathophysiology. In this study we have performed an association analysis of five polymorphic microsatellite markers in the three different NO synthase (NOS) genes; nNOS (NOS1), iNOS (NOS2A) and eNOS (NOS3). Ninety-one cluster headache patients diagnosed according to International Headache Society criteria and 111 matched controls were studied. Phenotype and allele frequencies were similarly distributed in patients and controls except for an iNOS (NOS2A) pentanucleotide repeat allele which was significantly more common in controls. We observed a higher phenotype frequency of this allele in our control group compared with rates in control groups of other studies, whereas the frequency in our patients was similar to that in controls from previous reports. Thus, we conclude that it is unlikely that genetic variations within the NOS genes contribute greatly to cluster headache susceptibility.     

Cytochrome-P450-dependent hydroxylation in cluster headache

Two isozymes of the cytochrome-P450-dependent drug oxidizing system exhibit polymorphism. Five to 10% of a Caucasian population are deficient in debrisoquine-hydroxylase activity and about 3% in mephenytoin-hydroxylase activity (poor metabolizers). We tested the hypothesis of a possible over-representation of poor metabolizers among patients with cluster headache. The individual metabolic capacity was determined in 30 cluster headache patients after administration of a test dose of 10 mg of debrisoquine and 100 mg of mephenytoin. Two patients (6.7%) were poor metabolizers of debrisoquine and one (3.3%) a poor metabolizer of mephenytoin. This was no different from the rate of poor metabolizers, 7.1% and 3.3% respectively, in a reference panel of healthy Swedish volunteers.