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Biomedical subjects

K Ekbom

Publications and source records attributed to K Ekbom.

At least 19 recordsLinked to original sources
PubMed2005

Familial cluster headache. Is atypical cluster headache in family members part of the clinical spectrum?

Familial cluster headache (CH) was analysed in 21 Swedish families. Diagnosis was made according to The International Classification of Headache Disorders 2004. We identified 55 affected, of whom 42 had episodic or chronic CH, one had probable CH and 12 had atypical symptoms. The atypical cases did not fulfil the diagnostic criteria for CH, but had clinical symptoms with more resemblance to CH than to migraine or other trigeminal autonomic cephalgia syndromes. The overall male : female ratio was 1.8:1. The overall mean age at onset was significantly lower in the second/third generation than in the first generation (mean age at onset 22 vs. 31 years, SD +/- 7 vs. 13 years; P < 0.01). This may be anticipation or selection bias, since individuals with late age at onset from the second/third generation may not yet have symptoms. The prevalence of migraine was 24% (13/55), i.e. similar to the prevalence in the general population. The high incidence of atypical CH cases in the Swedish families with other members affected with CH may suggest that the spectrum of CH is broader than previously thought. We suggest that atypical CH in CH families may represent an expanded spectrum of the disease with a common aetiology, i.e. a common genetic background.

Adult↗
PubMed2005

Nitrous oxide inhalation is a safe and effective way to facilitate procedures in paediatric outpatient departments.

AIMS: To evaluate the efficacy and safety of nitrous oxide treatment given to children presenting procedural problems in a paediatric outpatient department. METHODS: The study comprised 70 children 6-18 years old. Two different groups were studied. (1) Children presenting with problems in establishing venous cannulation (VC) (n = 50). The patients were randomised to conventional treatment (CO); cutaneous application of EMLA or nitrous oxide treatment (NO); N2O and EMLA. (2) Anxious children/children undergoing painful procedures who repeatedly come to the clinic (n = 20). These children underwent two procedures with CO/NO, the order of priority being randomised. Altogether the study included 90 procedures. Main outcome measures were procedure time, number of attempts required to establish VC, pain, and evaluation. RESULTS: All procedures were performed with NO while four VC (8%) were not possible to perform with CO. The number of attempts required to establish VC was lower when using NO (median 2, range 2-9), compared with CO (median 4, range 2-9). The estimated pain was lower with NO. The total mean time required was similar for NO and CO when the time required for the NO procedure was included. One complication, tinnitus, was observed; it disappeared within 3 minutes. CONCLUSION: The pretreatment with nitrous oxide is a time effective and safe method for use at paediatric outpatient departments to reduce pain, facilitate venous cannulation, and thereby reduce the number of costly cancellations of planned procedures.

Adolescent↗
PubMed2004

Periods of cluster headache induced by nitrate therapy and spontaneous remission of angina pectoris during active clusters.

Glyceryl trinitrate (GTN) is known to induce single extra attacks of cluster headache (CH) during active cluster periods, most probably via actions of nitric oxide (NO). Induction of whole periods of CH by organic nitrates has, however, attracted little attention in the literature. We report on eight patients with episodic CH and coexistent effort-induced angina pectoris. Cases 1-6 had been free of their headaches for many years but got recurrence of CH within a few weeks after the administration of long-acting organic nitrates (isosorbide-dinitrate, isosorbide-5-mononitrate or slow-release GTN) aimed at treating their chest pains. These nitrate-induced headache periods were more severe and had a longer duration than the previous spontaneous ones. Furthermore, one of the subjects and two additional cases experienced a marked reduction of their anginal attacks during successive CH periods. Exercise time to effort-induced angina was increased in all three patients and one of them revealed a markedly elevated threshold for eliciting ischaemic cardiac symptoms by standardized physical exercise on a cycle ergometer. We hypothesize whether extra CH periods elicited by sustained nitrate therapy and remission of angina pectoris during active clusters are caused by central mechanisms involving inhibition of sympathetic tone and effects on both cranial vessels and cardiac functions.

Adolescent↗
PubMed2003

A note on cluster headache in a population-based twin register.

Evidence of a familial risk factor in cluster headache is accumulating and studies of twin concordance may resolve family resemblance into genetic and environmental influences. The past literature on cluster headache in twins comprises a few case reports of concordant monozygotic pairs. Swedish twin pairs with a diagnosis of cluster headache were selected through a cross-match of national registers of twin births and hospitalizations. Seventeen discordant twin pairs were found, in which it was possible to verify cluster headache status in 11 complete pairs (two monozygotic, four dizygotic, and five unlike-sexed pairs). In both members of a female monozygotic pair, migraine without aura developed after birth of the first child and remitted by menopause, whereas post-menopausal development of chronic cluster headache occurred in only one of them. The importance of individual specific factors for cluster headache was demonstrated. However, to explain familial aggregation a larger sample of affected twin pairs is necessary.

Adult↗
PubMed2002

Age at onset and sex ratio in cluster headache: observations over three decades.

Five hundred and fifty-four patients with episodic cluster headache (ECH) and chronic cluster headache (CCH) were examined between 1963 and 1997. Mean age at onset was significantly higher in women with CCH compared with women with ECH and in men with ECH or CCH. In women with CCH age at onset was evenly distributed from 10 to 69 years, whereas in men with CCH and in both sexes with ECH, there was a peak when they were in their 20s. In women with ECH a second peak of onset occurred in their 50s. Although not statistically significant, primary CCH started later in women (mean 50.8 years) than secondary CCH (mean 35.5 years). There was a significant variation in the male : female ratio with respect to age at onset, being largest between 30 and 49 years of age (ECH 7.2 : 1; CCH 11.0 : 1) and lowest after 50 (ECH 2.3 : 1; CCH 0.6 : 1). During the observation period of more than 30 years there was a trend towards a decreasing male preponderance; the male : female ratio was significantly higher among patients with onset before rather than after 1970. The proportion of episodic vs. chronic CH did not change during the study period. The nature of the sex- and age-related pattern of cluster headache onset remains to be elucidated but mechanisms associated with sex hormone regulation, perhaps of hypothalamic origin, may be involved, as well as environmental factors related to lifestyle.

Adolescent↗
PubMed2002

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.

Adult↗
PubMed2001

CACNA1A gene polymorphisms in cluster headache.

Cluster headache (CH) is a primary headache disorder where the aetiological and pathophysiological mechanisms still are largely unknown. An increased risk of CH in first- and second-degree relatives suggests the importance of genetic factors. Mutations of the P/Q type calcium channel alpha 1 subunit (CACNA1A) gene on chromosome 19p13 have been shown to cause several neurological disorders with a wide clinical spectrum, mainly episodic diseases. Missense mutations of the gene cause familial hemiplegic migraine (FHM) and it is also likely to be involved in the more common forms of migraine. The CACNA1A gene is thus a promising candidate gene for CH. In this study we performed an association analysis of an intragenic polymorphic (CA)n-repeat with marker D19S1150 and a (CAG)n-repeat in the 3'UTR region, in 75 patients with CH according to IHS criteria and 108 matched controls. Genotypes and allele frequencies were similarly distributed in patients and controls. Linkage disequilibrium between the two markers was similar in patients and controls. We conclude that an importance of the CACNA1A gene in sporadic CH is unlikely.

Adult↗
PubMed2000

Cluster headache is not associated with signs of a systemic inflammation.

OBJECTIVE: To investigate whether there is clinical or biochemical evidence for a transient systemic inflammation during active periods of cluster headache. METHODS: Twenty-seven male and female consecutively selected patients with episodic cluster headache filled in questionnaires aiming at detecting any concurrent systemic vasculitic or rheumatoid disease. They were physically examined by both a neurologist and a rheumatologist independent of each other. Blood and urine samples were taken one to three times during an active cluster period and once in remission. The following analyses were performed: hemoglobin, erythrocyte sedimentation rate, C-reactive protein, complete blood counts including differential counts, creatinine, albumin, creatine kinase, electrophoreses of serum (with haptoglobin, orosomucoid, IgG, IgM), von Willebrand's factor, antinuclear antibodies, rheumatoid factor, cytoplasmic antineutrophil cytoplasmic autoantibodies, perinuclear antineutrophil cytoplasmic autoantibodies, and routine urinary tests. An age- and sex-matched control group of 99 consecutive patients attending the Outpatient Department of Neurology for symptoms/diseases other than severe headache completed the same questionnaire as the patient group. RESULTS: Only one patient with cluster headache showed clinical signs (livedo reticularis) that could have been due to an ongoing systemic vasculitis. Most symptoms were equally or even more prevalent in the control group than among the patients with cluster headache. However, cold feet were about twice as prevalent among female patients with cluster headache than in the control group. This was considered due to their smoking habits. Laboratory tests showed no statistically significant differences between the active cluster periods and remission. There were some slightly abnormal values in single laboratory tests, some of which were probably due to concurrent upper respiratory infections. The findings of laboratory tests for one patient could have been due to nephritis. All patients were negative for cytoplasmic antineutrophil cytoplasmic autoantibodies and perinuclear antineutrophil cytoplasmic autoantibodies. CONCLUSIONS: These results were taken as evidence that no systemic inflammation is present during the active cluster headache period. However, whether a local retro-orbital inflammation underlies the pathophysiology of cluster headache remains obscure.

Adolescent↗
PubMed2000

A follow-up study of 60 patients after an assumed first period of cluster headache.

During 1981-96 a series of 60 consecutive out-patients was examined in relation to an assumed first period of cluster headache (CH). On follow up in 1998 we found that six were deceased at a mean age of 56.5 years (range 45-74 years), of whom one had a definitive CH diagnosis and five had one documented headache period only. Six patients were lost to follow up because they could not be reached. In the final group for evaluation (n = 49) it was found that 13 (26.5%) patients had had one cluster period only during a mean observation time of 8.9 years. Out of 36 patients with a definitive CH diagnosis according to International Headache Society (IHS) criteria, 31 patients had episodic CH, four patients had primary chronic CH and one patient had secondary chronic CH. Of the patients with a definitive CH diagnosis, 83% on follow up had had a recurrence of a second period of CH within 3 years or continuous attacks (chronic/semichronic CH) from the onset. Evidently some patients may suffer from one cluster period only. In our patient material only 17% had a second cluster period after 3 years.

Adult↗
PubMed1995

Cluster headache attacks treated for up to three months with subcutaneous sumatriptan (6 mg). Sumatriptan Cluster Headache Long-term Study Group.

In the first three months of a 24-month open study to assess the safety and efficacy of subcutaneous sumatriptan 6 mg in the long-term acute treatment of cluster headache, 138 patients treated a maximum of two attacks daily each with a single 6 mg injection. A total of 6353 attacks were treated. Adverse events, reported in 28% of sumatriptan-treated attacks, were qualitatively similar to those seen in migraine long-term trials. Their incidence did not increase with frequent use of sumatriptan. There were no clinically significant treatment effects on vital signs, ECG recordings or laboratory parameters. Headache relief (a reduction from very severe, severe or moderate pain to mild or no pain) at 15 min was obtained for a median of 96% of attacks treated. There was no indication of tachyphylaxis, decrease in the speed of response, or increased frequency of attacks with long-term treatment. This study demonstrated that, in long-term use, subcutaneous sumatriptan 6 mg is a well-tolerated and effective acute treatment for cluster headache.

Adolescent↗
PubMed1995

Migraine, chronic tension-type headache, and cluster headache in an Ethiopian rural community.

Fifteen-thousand-five-hundred adults (> or = 20 years) in a rural district in Ethiopia with a population of 250,000 were studied for chronic headache. Door-to-door survey was performed by trained lay health workers using a questionnaire with a high degree or reliability and validity. The 1-year prevalence of migraine headache was 3.0% (4.2% for females and 1.7% for males) with the peak age specific rate in the fourth decade. Migraine headache was about three times more common in females than in males at any decade. Two-thirds of migraine sufferers had rather frequent attacks. The most frequent trigger factors were emotional stress (90%), changes of weather (78%), physical exhaustion (75%), and smell (70%). Migraine with aura was rare. Family occurrence of migraine in first-degree relatives was 30%. The 1-year prevalence of chronic tension-type headache was 1.7%, while cluster headache was found to be extremely rare. Compared to similarly performed surveys, the prevalence of migraine among rural Ethiopians was less than among Nigerian Africans.

Adult↗
PubMed1995

Treatment of cluster headache: clinical trials, design and results.

The spontaneous capricious course of cluster headache may give rise to some problems when treatment is being evaluated. This is one of several explanations for there being so few well-designed, randomized, double-blind clinical trials in cluster headache. The standard treatment of acute attacks of cluster headache is inhalation of 100% oxygen. In the prophylaxis of episodic cluster headache, ergotamine, verapamil, lithium, serotonin, inhibitors and steroids are used. In chronic cluster, lithium is the drug of choice, but verapamil may also be tried. Recently, hyperbaric oxygen has been shown to immediately abort acute attacks, and it seems that it may also be useful in the prophylactic treatment. The introduction of the novel 5HT1 agonist sumatriptan as a symptomatic relief of cluster attacks represents further significant progress. Two randomized, double-blind, placebo-controlled, cross-over trials have shown sumatriptan 6 mg sc to be a rapid, effective and well-tolerated acute treatment for cluster headache attacks. Within 15 min of treatment, 74% of attacks on sumatriptan responded compared to 26% of placebo-treated attacks. Functional disability was also significantly improved. Increasing the dose to 12 mg did not offer significantly greater relief compared to sumatriptan 6 mg, but was associated with an increased incidence of adverse events. Interim analysis of 3 months of data from a recent multinational open trial comprising, 138 patients having treated 6353 attacks with subcutaneous sumatriptan 6 mg revealed a headache relief in 96% of attacks treated. There was no evidence of an increased incidence of adverse events with frequent use of sumatriptan. No tachyphylaxis was seen over the 3 months, suggesting that sumatriptan is effective and well tolerated also in long-term acute treatment for cluster headache.

Adolescent↗
PubMed1995

Ekbom's syndrome of photomyoclonus, cerebellar ataxia and cervical lipoma is associated with the tRNA(Lys) A8344G mutation in mitochondrial DNA.

We have investigated nine maternal offsprings to patients with a hereditary syndrome of cerebellar ataxia, photomyoclonus, skeletal deformities and lipoma, originally described by Ekbom. The nine family members underwent a thorough neurological examination, neurophysiological investigations and molecular genetic analysis of mtDNA from lymphocytes and muscle. Clinical examination showed a partial syndrome in one relative and minor signs and symptoms in three additional offsprings. We found the heteroplasmic tRNALys A8344G point mutation in mtDNA in all investigated maternal offsprings. The fraction of mutated mtDNA ranged from 33 to 87% in lymphocytes and from 59 to 92% in muscle tissue. Analysis of mtDNA from a lipoma showed a high level (96%) of the tRNALys A8344G mutation. We conclude that Ekbom's syndrome is a mitochondrial encephalomyopathy associated with the same heteroplasmic tRNA mutation as seen in myoclonus epilepsy with ragged-red fiber (MERRF) syndrome.

Adult↗
PubMed1994

Clinical experiences from Sweden on the use of subcutaneously administered sumatriptan in migraine and cluster headache.

This article reviews, from the practitioner's point of view, more than 1 year of clinical experience of the use of subcutaneously administered sumatriptan succinate in the short-term treatment of migraine and cluster headache with regard to advantages and disadvantages of the drug. In accordance with the results of clinical trials, subcutaneous sumatriptan, also in the practitioner's hands, was found to relieve migraine headaches and all other symptoms associated with migraine in most patients and within a reasonable period. Adverse events, however, are common and were perceived by about 70% of the patients. The most common adverse events were pressure/stiffness in the neck and throat (32%), general tiredness (22%), pressure/tightness over the chest (21%), injection site reactions (16%), and tingling sensations in the head and arms (14%). Headache recurrence within 24 hours is a clinical problem not only for the patient but also for the prescribing physician. About every second (53%) migraineur using subcutaneous sumatriptan reports headache recurrence. Headache recurrence appears to be effectively treated by a second injection. Pending valid information about effects, adverse events, headache recurrence, and how to handle the autoinjector, the compliance and tolerability of subcutaneous sumatriptan appear to be most satisfactory among eligible patients with migraine.

Cardiovascular Diseases↗