Diagnostic and therapeutic errors in cluster headache: a hospital-based study.

BACKGROUND: Cluster headache (CH) is a severe, disabling form of headache. Even though CH has a typical clinical picture it seems that its diagnosis is often missed or delayed in clinical practice. CH patients may thus face: misdiagnosis, unnecessary investigations and delays in accessing adequate treatment. This study was conducted to investigate the occurrence of diagnostic and therapeutic errors with a view to improving the clinical and instrumental work-up in affected patients. METHODS: Our study comprised 144 episodic CH patients: 116 from Italy and 28 from Eastern European countries (Moldova, Ukraine, Bulgaria). One hundred six patients (73.6%) were examined personally and 38 (26.4%) were evaluated through telephone interviews conducted by headache specialists using an ad hoc questionnaire developed by the authors. RESULTS: The sample was predominantly male (M:F ratio 2.79:1) and had a mean age of 42.4 ± 9.8 years; approximately 76% of the patients had already consulted a physician about their CH at the onset of the disease. The mean interval between onset of the disease and first consultation at a headache center was 4.1 ± 5.6 years. The patients had consulted different specialists prior to receiving their CH diagnosis: neurologists (49%), primary care physicians (35%), ENT specialists (10%), dentists (3%), etc. Misdiagnoses at first consultation were recorded in 77% of the cases: trigeminal neuralgia (22%), migraine without aura (19%), sinusitis (15%), etc. The average "diagnostic delay" was 5.3 ± 6.4 years and the condition was diagnosed approximately ("doctor delay": one year). Instrumental and laboratory investigations were carried out in 93% of the patients prior to diagnosis of CH. Some of the patients had never received abortive or preventive medications, either before or after diagnosis. Medical prescription compliance: 88% of the cases. CONCLUSIONS: Our results emphasize the need to improve specialist education in this field in order to improve recognition of the clinical picture of CH and increase knowledge of the proper medical treatments for de novo CH. Continuous medical education on CH should target general neurologists, primary care physicians, ENT specialists and dentists. A study on a larger population of CH patients may further improve error-avoidance strategies.

Diagnosis and clinical features of trigemino-autonomic headaches.

Although severe short-lasting headaches are rare, they can be considered disabling conditions with a major impact on the quality of life of patients. These headaches can divided broadly in to those associated with autonomic symptoms, so called trigeminal autonomic cephalgias (TACs), and those with few or no autonomic symptoms. The TACs include cluster headache, paroxysmal hemicranias, hemicrania continua, and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms as well as short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome. In all of these syndromes, half-sided head pain and ipsilateral cranial autonomic symptoms such as lacrimation or rhinorrhea are prominent. The paroxysmal hemicranias have, unlike cluster headaches, a very robust response to indomethacin, leading to a notion of indomethacin-sensitive headaches. The diagnosis of TACs is exclusively a clinical task. Because of the fact that cluster headache is strictly half-sided, typically involves the region around the eye and temple and often starts in the upper jaw, most patients first consult a dentist or ophthalmologist. No single instrumental examination has yet been able to define, or ensure, the correct diagnosis, or differentiate idiopathic headache syndromes. It is crucial that a trained neurologist sees these patients early so that management can be optimized and unnecessary procedures can be avoided. Although TACS are, in comparison to migraine, quite rare, they are nevertheless clinically very important for the neurologist to consider as they are easy to diagnose and the treatment is very effective in most patients.

Clinical course of patients with episodic cluster headache treated with corticosteroids inproximity to the sphenopalatine ganglion: a preliminary study of 23 patients.

OBJECTIVE: A study is made of the clinical course of patients with episodic cluster headache following the injection of corticosteroids in the proximity of the sphenopalatine ganglion of the affected side. STUDY DESIGN: A retrospective observation study was made corresponding to the period between 2006 and 2010. Patients with episodic cluster headache received corticosteroid infiltrations in the vicinity of the sphenopalatine ganglion. Data were collected to assess the clinical course, quantifying pain intensity and quality of life. A total of 23 patients (11 women and 12 men) with a mean age of 50.4 years (range 25-65) were included. Forty percent of the patients had undergone dental extractions in the quadrant affected by the pain, before the development of episodic cluster headache, and 37.8% underwent extractions in the same quadrant after appearance of the headache. RESULTS: Most of the patients suffered 1-3 attacks a day, with a duration of pain of between 31-90 minutes. The mean pain intensity score during the attacks at the time of the first visit was 8.8 (range 6-10), versus 5.4 (range 3-9) one week after the first corticosteroid injection. On the first visit, 86.9% of the patients reported unbearable pain, versus 21.7% after one week, and a single patient after one month. CONCLUSIONS: The evolution of episodic cluster headache is unpredictable and variable, though corticosteroid administration clearly reduces the attacks and their duration.

5-HT(1D) receptor immunoreactivity in the sphenopalatine ganglion: implications for the efficacy of triptans in the treatment of autonomic signs associated with cluster headache.

OBJECTIVE: To determine if 5-HT(1D) receptors are located in the sphenopalatine ganglion. BACKGROUND: While the 5-HT(1D) receptor has been described in sensory and sympathetic ganglia in the head, it was not known whether they were also located in parasympathetic ganglia. METHODS: We used retrograde labeling combined with immunohistochemistry to examine 5-HT(1D) receptor immunoreactivity in rat sphenopalatine ganglion neurons that project to the lacrimal gland, nasal mucosa, cerebral vasculature, and trigeminal ganglion. RESULTS: We found 5-HT(1D) receptor immunoreactivity in nerve terminals around postganglionic cell bodies within the sphenopalatine ganglion. All 5-HT(1D) -immunoreactive terminals were also immunoreactive for calcitonin gene-related peptide but not vesicular acetylcholine transporter, suggesting that they were sensory and not preganglionic parasympathetic fibers. Our retrograde labeling studies showed that approximately 30% of sphenopalatine ganglion neurons innervating the lacrimal gland, 23% innervating the nasal mucosa, and 39% innervating the trigeminal ganglion were in apparent contact with 5-HT(1D) receptor containing nerve terminals. CONCLUSION: These data suggest that 5-HT(1D) receptors within primary afferent neurons that innervate the sphenopalatine ganglion are in a position to modulate the excitability of postganglionic parasympathetic neurons that innervate the lacrimal gland and nasal mucosa, as well as the trigeminal ganglion. This has implications for triptan (5-HT(1D) receptor agonist) actions on parasympathetic symptoms in cluster headache.

Essential oils and cluster headache: insights from two cases.

Essential oils with proconvulsive properties are known to cause seizures and may worsen migraine. Here, we report two cases of cluster headache temporally related to the use of toothpastes containing essential oils of camphor and eucalyptus.

[Atypical facial pains--sluder's neuralgia--local treatment of the sphenopalatine ganglion with phenol--case report]. / Nietypowe bóle twarzy--neuralgia sludera-- leczenie miejscowe--przyieganie zwoju skrzydlowo-- podniebiennego fenolem--opis przypadków.

AIM: Chronic reccuring head and facial pain can be very difficult for successful treatment. Such a pain can be in some rare cases Sluder's sphenopalatine ganglion neuralgia. The aim of the study was to obtain the pain relief by local treatment in patients with Sluder's sphenopalatine ganglion neuralgia. METHODS: We described three cases of Sluder's neuralgia among all the seventeen patients with reccuring head and face pain that were seen in our department. In all these cases 4% Xylocaine was applied intranasally, into the region of shenopalatine ganglion, behind the posterior tip of the middle turbinate four times for ten minutes. According to Kern, the diagnosis of Sluder's neuralgia was confirmed only in cases where local anesthetic block of the sphenopaltine ganglion was successful. It means the patients were pain-free for at least an hour after application of Xylocaine, so they were qualified for phenolization and 88% phenol was applied on the cotton carriers (number of the applications depended on the patient). RESULTS: The total relief of pain of different duration was obtained in all the presented cases. CONCLUSION: The relief of pain obtained by intranasal phenolization of sphenopalatine ganglion in three patients shows it could be the effective treatment of Sluder's neuralgia. The patients were totally free from the pain and accompanying symptoms like nasal obstruction, rhinorrhea, epiphora or conjunctivitis. The relief period was different but the patients were satisfied with the effectiveness and simplicity of the treatment. They did not need to take the additional medications for months and were able to continue work.

Sumatriptan. An updated review of its use in migraine.

UNLABELLED: Sumatriptan is a selective agonist at serotonin 5-HT1-like receptors, including 5-HT1B/1D subtypes. It is an effective treatment for acute migraine attacks and the injectable form has also shown efficacy in the treatment of cluster headaches. In placebo-controlled clinical trials, sumatriptan, administered subcutaneously, orally, intranasally or rectally was significantly more effective than placebo in relieving migraine headache and in producing resolution or reduction of other symptoms associated with migraine, including nausea, photophobia and phonophobia. Improvements in clinical disability were also significantly greater after sumatriptan than after placebo. Headache recurred in 21 to 57% of patients who received oral or subcutaneous sumatriptan, but most patients responded to a second dose of the drug. Results of comparative trials showed that subcutaneous sumatriptan 6 mg was significantly more effective than either patients' usual antimigraine treatments or intranasal dihydroergotamine mesylate 1 mg in relieving migraine headache. Subcutaneous sumatriptan 6 mg and subcutaneous dihydroergotamine mesylate 1 mg provided similarly effective migraine relief, but the headache recurrence rate was significantly higher after sumatriptan than after this formulation of dihydroergotamine mesylate. Response rates achieved after oral sumatriptan were similar to those reported after treatment with oral naratriptan, rizatriptan or lysine acetylsalicylate plus metoclopramide. Treatment of acute migraine attacks with oral or subcutaneous sumatriptan leads to less loss of workplace productivity than other antimigraine therapies. Several pharmacoeconomic analyses showed that gains in workplace productivity in sumatriptan recipients ranged from 12.1 to 89.8 hours per patient per year. Significant improvements from baseline in overall health-related quality-of-life scores were also experienced by sumatriptan recipients. Sumatriptan is generally well tolerated. Nausea, vomiting, malaise and fatigue are the most common adverse events with oral sumatriptan. Injection site reactions occur in 10 to 40% of patients receiving the drug subcutaneously. A bitter taste at the back of the mouth occurs frequently after intranasal administration. Serious adverse events occur in about 0.14% of patients with migraine treated with sumatriptan. As the drug is associated with the rare development of cardiovascular effects, it is contraindicated in patients with a history of cardiovascular disease. CONCLUSIONS: Despite its relatively high acquisition cost, reductions in lost workplace productivity experienced by patients treated with sumatriptan may result in savings in the overall cost of migraine to society. Thus, sumatriptan is a useful first- or second-line treatment option for patients with moderate or severe migraine.

Efficacy of sphenopalatine ganglion blockade in 66 patients suffering from cluster headache: a 12- to 70-month follow-up evaluation.

This study was conducted to evaluate the efficacy, based on 12- to 70-month follow-up data, of radiofrequency (RF) lesions of the sphenopalatine ganglion made in patients suffering from cluster headache. Sixty-six patients suffering from either episodic (Group A, 56 patients) or chronic (Group B, 10 patients) cluster headache who were not responsive to pharmacological management were treated by RF lesioning in the sphenopalatine ganglion. Complete relief of pain was achieved in 34 (60.7%) of 56 patients in Group A and in three (30%) of 10 patients in Group B. No relief was found in eight patients (14.3%) in Group A and in four (40%) in Group B. The mean time of follow up was 29.1 +/- 10.6 months in Group A and 24 +/- 9.7 months in Group B, ranging from 12 to 70 months. With regard to side effects and complications, temporary postoperative epistaxis was observed in eight patients and a cheek hematoma in 11 patients; a partial RF lesion of the maxillary nerve was inadvertently made in four patients. Nine patients complained of hypesthesia of the palate, which disappeared in all cases within 3 months. The authors conclude that RF lesioning in the sphenopalatine ganglion via the infrazygomatic approach may be performed in patients suffering from cluster headache that does not respond to pharmacological therapy.

Episodic cluster headache in a community: clinical features and treatment.

OBJECTIVE: To study the clinical features and treatment given to episodic cluster headache patients in the Calgary region. PATIENTS: Fifty-one (51) patients who responded to a media campaign, had previously been diagnosed by their family physicians, and who met International Headache Society (IHS) criteria for episodic cluster headache, formed the population for this study. METHODS: The media campaign consisted of newspaper advertisements and radio publicity including physician interviews and talk shows. Patients were required to complete by 200-item questionnaire detailing clinical features and treatment of their cluster headache syndrome. Each patient was also interviewed by our research nurse for clarification and proper completion of questionnaire. RESULTS: Fifty-one percent (51%) of our patients had short headache attacks lasting one hour or less. Almost one-half (45%) had three or four attacks per 24 hour period. Eighty-six percent (86%) had been referred to a neurologist. Sixty-nine percent (69%) had never used oxygen, but of those who had, one-half were still using it. Sumatriptan by injection had been tried by 26% of patients and of these, 93% considered it effective. Subcutaneous dihydroergotamine had been tried by 8%. For prophylaxis, 41% had tried methysergide, 31% prednisone, and 4% verapamil. Many patients had been prescribed migraine prophylactic drugs which are ineffective for cluster headache, and some had also undergone dental procedures or nasal and sinus surgeries. CONCLUSIONS: Many cluster headache patients had not, to their knowledge, been prescribed or used the best symptomatic and prophylactic treatments for cluster headache. This should be addressed through educational programs and through making up-to-date information on the treatment of cluster headache readily available to physicians and patients.

Cluster headache following dental treatment: a case report.

Cluster headache is a neurovascular disorder characterized by attacks of severe and strictly unilateral pain presenting in and around the orbit and temporal area. Attacks occur in series lasting for weeks or months separated by remission periods. An individual attack lasts 15-180 min with a frequency of once every other day to as often as 8 times per day. Ipsilateral radiation of the headache to orofacial regions, including the teeth, is not unusual. The area of involvement may obscure the diagnosis and lead to irreversible and unnecessary dental treatment. A case in which cluster attacks occurred immediately after a dental procedure is described.

Verapamil induced gingival enlargement in cluster headache.

Verapamil is an effective prophylactic treatment for cluster headaches and, therefore, is widely used. This report describes four patients with cluster headache who developed gingival enlargement after initiating treatment with verapamil. In two patients, it was possible to control this side effect adequately by optimising oral hygiene and dental plaque control. In the other two patients, lowering of the verapamil dose, in addition to optimal oral hygiene and dental plaque control, was necessary; in one patient verapamil had to be stopped completely to reverse the gingival enlargement. Doctors treating cluster headache with verapamil need to be aware of this side effect, especially as it may be preventable with good dental hygiene and dental plaque control, is reversible with reduction or cessation of verapamil, and can lead to dental loss.

Antiepileptic drugs in the management of cluster headache and trigeminal neuralgia.

Cluster headache and trigeminal neuralgia are relatively rare but debilitating neurologic conditions. Although they are clinically and diagnostically distinct from migraine, many of the same pharmacologic agents are used in their management. For many patients, the attacks are so frequent and severe that abortive therapy is often ineffective; therefore, chronic preventive therapy is necessary for adequate pain control. Cluster headache and trigeminal neuralgia have several distinguishing clinical features. Cluster headache is predominantly a male disorder; trigeminal neuralgia is more prevalent in women. Individuals with cluster headaches often develop their first attack before age 25; most patients with trigeminal neuralgia are between age 50 and 70. Cluster headaches are strongly associated with tobacco smoking and triggered by alcohol consumption; trigeminal neuralgia can be triggered by such stimuli as shaving and toothbrushing. Although the pain in both disorders is excruciating, cluster headache pain is episodic and unilateral, typically surrounds the eye, and lasts 15 to 180 minutes; the pain of trigeminal neuralgia lasts just seconds and is usually limited to the tissues overlying the maxillary and mandibular divisions of the trigeminal nerve. Cluster headache is unique because of its associated autonomic symptoms. Although the pathophysiology of cluster headache and trigeminal neuralgia are not completely understood, both appear to have central primary processes, and these findings have prompted investigations of the effectiveness of the newer antiepileptic drugs for cluster headache prevention and for the treatment of trigeminal neuralgia. The traditional antiepileptic drugs phenytoin and carbamazepine have been used for the treatment of trigeminal neuralgia for a number of years, and while they are effective, they can sometimes cause central nervous system effects such as drowsiness, ataxia, somnolence, and diplopia. Reports of studies in small numbers of patients or individual case studies indicate that the newer antiepileptic drugs are effective in providing pain relief for trigeminal neuralgia and cluster headache sufferers, with fewer central nervous system side effects. Divalproex has been shown to provide effective pain control and to reduce cluster headache frequency by more than half in episodic and chronic cluster headache sufferers. Topiramate demonstrated efficacy in a study of 15 patients, with a mean time to induction of cluster headache remission of 1.4 weeks (range, 1 day to 3 weeks). In the treatment of trigeminal neuralgia, gabapentin has been shown to be effective in an open-label study. When added to an existing but ineffective regimen of carbamazepine or phenytoin, lamotrigine provided improved pain relief; it also may work as monotherapy. Topiramate provided a sustained analgesic effect when administered to patients with trigeminal neuralgia. The newer antiepileptic drugs show considerable promise in the management of cluster headache and trigeminal neuralgia.