Abstract
Introduction
Although migraine and cluster headache are two distinct and different entities, occasionally some patients can present with characteristics of both headache disorders occurring simultaneously during attacks (1–3). Either migrainous features can be seen in a patient with cluster headache (1, 3), or unilateral cranial autonomic symptoms can be seen in a patient with typical migraine headache (2, 3), or features of both can be mixed together without one being predominant (3). We present a case with migraine and cluster-like features such as cranial autonomic symptoms and strict unilaterality.
Case report
A 27-year-old female medical professional presented with a 8-year history of episodic headaches. She reported that her headaches used to occur once to twice monthly but had increased in frequency during the last 6 months, now occurring up to three times a week. The attacks never followed a special rhythm but always appeared randomly. The headache usually built up over half an hour and used to be very intense for 1–2 h, but this was always followed by a more bearable pain lasting for 24 h which was usually abolished if she could manage to sleep. In the early years the headache used to occur during night time, waking her up, usually 2–3 h after she went to sleep, but during the last 3–4 years it could happen at any time of the day.
The pain was strictly localized to the left frontotemporal region and of pulsatile quality. It was increased by physical activity and bending forwards. Nausea/vomiting, photophobia and phonophobia always accompanied the pain. During the pain she experienced lacrimation, conjunctival injection and ptosis in the left eye. She mentioned that the pain was usually precipitated when she stayed in stuffy rooms, especially with cigarette smoke. Although she seldom use alcoholic beverages, she reported that the headache was precipitated once or twice when she had had a few glasses of white wine, but she could not exactly remember how long after ingestion the headache started.
During an attack she preferred to rest in a quiet, dark room. She had used non-steroidal anti-inflammatory drugs for her attacks several times but they were of no use. She responded well to ergotamine preparations and sumatriptan. She tried oxygen a few times, at a time her attacks had become very infrequent after we had started treatment with verapamil. She reported that with oxygen her attacks were shortened and only a mild ache persisted for some hours. She had never tried oxygen before she came to our clinic, as she had always been diagnosed as migraine headache. Previous prophylactic medication trials included pizotifen which she had used for 3 months and had responded well, and magnesium and propranolol, to which she did not respond at all, but she reported to have used propranolol for only 1 month.
She did not report any family history of migraine or of cluster headache.
Neurological examination, blood tests, EEG, magnetic resonance imaging and MR angiogram were all normal.
The patient was started on verapamil 160 mg/day. She responded well to treatment and after 2 weeks she reported having only two attacks. Attacks were completely abolished after 2 months of treatment.
Discussion
Cluster headache, which was classified as a migraine variant in the 1950s (4), has since been identified as a distinct and separate headache disorder. A history of migraine is not uncommon in patients with cluster headache, and both headache disorders have been reported to coexist non-simultaneously in the same patient in more than a quarter of cases (5, 6). However, only occasionally do patients present with features of both migraine and cluster headache occurring simultaneously, and therefore Solomon et al. proposed the term ‘migraine–cluster headache syndrome’ (3).
Migraine headaches are known to switch sides even if they preferentially occur on one side, but during the past years cases with so-called ‘side locked migraine’ have been reported (2, 7, 8). Although the strict unilaterality and accompanying autonomic symptoms in our patient were more typical of cluster headache, there were many features, including the time pattern of occurrence, which better fitted the diagnosis of migraine. Cluster attacks seldom last more than 3 h and usually occur in bouts which last around 8 weeks (6). Although in our patient the duration of the intense pain never exceeded 2 h, the duration of the attack was usually 24 h, which raises the question whether both entities occurred simultaneously. The random occurrence of attacks was also more typical of migraine than episodic or chronic cluster headache. Patients with cluster headache are usually restless during an attack and unwilling to lie down as this results in increased pain (6, 9). In contrast, migraine patients usually prefer to lie down in a dark, quiet room and try to sleep, as this frequently ends the attack (10). Sleep relieves headache in migraineurs but has little impact on cluster headache (6, 9, 10). These features were all present in our patient. Nausea, vomiting, photophobia and sonophobia are all features which can be frequently seen in both headache disorders, and therefore are not valuable in distinguishing between migraine and cluster headache (6).
It could be debated whether this is a migraine with cluster features or a cluster headache with a migrainous pattern, but we thought that there were more characteristics of migraine than of cluster headache. Unilateral autonomic symptoms have been reported to occur in a large proportion of migraineurs in a study by Barbanti et al. (2). They concluded that the presence of unilateral autonomic symptoms suggests an activation of the trigeminal-autonomic reflex. Strictly unilateral migraine has been reported to be frequently associated with cranial autonomic symptoms (2, 7), which makes a discrimination from cluster headache quite difficult. Migraine and cluster headache have also been reported to coexist, although in different periods of life (5). On the other hand, reports of hemiplegic cluster, clinically similar to hemiplegic migraine, also exist, raising the question whether cluster headache is also a channelopathy (11).
Although migraine and cluster headache are considered two different entities with different, but not yet fully understood, pathophysiology, it is possible that they both share a common pathophysiological step, probably a functional alteration in hypothalamic or brainstem circuits.