Abstract
Introduction
Cluster headache is a primary headache syndrome occurring in attacks of unilateral, orbital, supraorbital and/or temporal pain of severe intensity. According to the criteria of the International Headache Society (IHS), at least one of the following signs has to be present on the pain-side: conjunctival injection, lacrimation, nasal congestion, rhinorrhea, forehead and facial sweating, miosis, ptosis, eyelid oedema. Each pain attack lasts 15–180 min and the pain attacks occur during cluster periods with a frequency of two–three per day. Symptomatic headaches must be excluded and the neurological examination is normal (1).
The exact pathophysiology of cluster headache is unknown. A peripheral neurogenic inflammatory process at the cavernosus sinus (2) and a central autonomic dysfunction within the regulation of the hypothalamic pituitary adrenal axis are considered to contribute to the pathogenesis of cluster headache (3).
For the treatment of the acute attack, 5HT1 receptor agonists, oxygen inhalation or (dihydro) ergotamine are used (4). For short-term prophylaxis corticosteroids and calciumantagonists are used, for long-term prophylaxis calciumantagonists and/or lithium can be used (5). Gabapentin (6) or topiramat (7) may be an effective alternative treatment.
We report a case with headache symptoms typical for cluster headache without any autonomic symptoms, responding to rizatriptan and corticosteroid treatment.
Case report
A 32-year-old male patient first visited our headache outpatient clinic in October 2000. For 3 days he had been suffering from attacks of strictly right-sided, supra- and periorbital headaches. The pain started mildly, reached its maximum within 20 min and released suddenly after 30–60 min. The pain intensity was described as very severe, reaching 8 at the numeric analogue scale from 1 to 10, and pain characteristic was described as burning and stabbing. The first two attacks occurred on 2 consecutive days during the afternoon, the third attack occurred during the night, awakening the patient.
As accompanying symptoms, only mild nausea was reported. No nasal congestion, rhinorrhea, conjuctival injection, lacrimation, miosis, ptosis, eyelid oedema or sweating was noticed by the patient. Motor hyperactivity was described. Triggers were denied. The patient took an analgesic compound containing acetylsalicylic acid and paracetamol without any effect. Unfortunately we did not see the patient during an attack, but we have investigated the patient with a very detailed structured interview.
Four years ago, the patient had suffered for 2 weeks from pain attacks with the same characteristics, which he thought were related to private stress. Since those attacks remitted spontaneously the patient did not see a doctor. Thirteen years ago he suffered from an idiopathic paresis of the left facial nerve, otherwise he had always been healthy. His family history was negative for headache.
On neurological examination the patient showed normal findings except a very mild deficit in the left third branch of the facialis nerve. Laboratory examinations showed a mild leukocytosis and slightly increased GPT and gGT. Intracranial tumours or vascular lesions were excluded by normal MRI findings. The ophthalmological examination, including measurement of ocular tension and a transcranial Doppler ultrasound, were normal.
Despite the lack of typical cluster-like autonomic features, an intravenous therapy with corticosteroids was started: 500 mg prednisolone for 3 days, 250 mg and 125 mg on the 4th and 5th day, respectively; in addition pantoprazol was administered at a dosage of 40 mg per day. For treating the acute attacks, rizatriptan 10 mg quick-solve tablets were prescribed; a self-administration of subcutaneous sumatriptan was refused by the patient. After corticosteroid treatment only two attacks occurred, which resolved 15 min after taking rizatriptan (10 mg).
Discussion
We report a case of episodic, strictly unilateral, periorbital and very severe headache attacks without autonomic symptoms. For differential diagnosis the following diseases were considered: sinusitis, glaucoma and other symptomatic headaches, migraine, upper cervical syndrome, cranial and facial neuralgia's, Reader neuralgia, Tolosa–Hunt-syndrome, chronic paroxysmal hemicrania, SUNCT syndrome and cluster headache (8).
Symptomatic headaches, especially alterations in the area of the sinus cavernosus, could be excluded by MRI. Migraine could be excluded clinically by the pain character, especially the short duration and frequency of the attacks and the lack of vegetative symptoms. Arguments against cranial and facial neuralgia's were the long duration of the attacks and the lack of any triggers, arguments against cervical syndrome were the periodicity of pain and no increase of symptoms during manipulations of the cervical spine. SUNCT syndrome and chronic paroxysmal hemicrania were excluded due to the course of the attacks, especially due to the relatively low frequency and longer duration of the attacks.
The patient was diagnosed as having cluster headache for the following reasons: unilateral, supra- and periorbital very severe pain occurring periodically in attacks, even during sleep. We considered the duration of the attacks, the sudden onset and remission, a remission of 4 years between two pain periods and the motor hyperactivity as typical for cluster headache. In addition, the patient responded to corticosteroids and 5HT1 agonists.
Although the IHS criteria (1) for cluster headache were not fulfilled as autonomic signs were completely missing, we thought the circadian rhythm and the clinical symptoms were typical for cluster headache and therefore started with corticosteroids and a 5HT1 agonist.
Other authors have reported indications for cluster headache without headache (9) as well as cluster headache without autonomic symptoms (10, 11). This is a more detailed case history of one patient with headache symptoms typical for cluster headache but without any autonomic symptoms.
We must observe whether the patient develops autonomic symptoms during time.