Abstract
Trigeminal autonomic cephalalgias (TACs) are characterized by severe, strictly unilateral pain in the territory of the distribution of the trigeminal nerve in association with autonomic manifestations (1). The International Headache Society (IHS) classification divides strictly unilateral headaches into three groups: cluster headache (CH), episodic and chronic paroxysmal hemicrania (PH), and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (1).
Symptomatic TACs are not common. However, they have been reported in association with the lesions of the sellar or parasellar structures, internal carotid artery dissection and vertebro-basilar artery diseases (1, 2). As for the disorders involving the vertebral artery, a case of a vertebral artery aneurysm and some cases of vertebral artery dissections (VADs) with lateral medullary infarctions have been reported (3–6).
To the best of our knowledge, there has not been any case report of VAD without infarction that was manifested by cluster-like headache. We describe here a case of a spontaneous VAD that presented with cluster-like headache without other neurological manifestations.
Case report
A 48-year-old woman suffering from severe headache in the left periorbital area and forehead visited our clinic. She had no prior history of primary headache, neurological diseases, or recent trauma. Her headache had started from the left posterior neck 10 days earlier. On the next day, it migrated forward and was localized over the left forehead and periorbital area. The nature of headache was of stabbing quality with abrupt onset and gradual offset. Each attack occurred several times a day, and lasted approximately 30 min. During the attack, she could not do anything and was restless because of severe headache. The headache was accompanied by ipsilateral tearing, red conjunctiva and eyelid swelling. Non-steroidal anti-inflammatory drugs reduced the initial posterior neck pain, but they did not relieve the anterior facial pain. Physical and neurological examinations were normal except for the left red conjunctiva and swollen eyelid. Laboratory tests, including complete blood counts, erythrocyte sedimentation rate, blood chemistry and electrolytes, were normal. She was initially diagnosed with episodic CH and subsequently administered 100% oxygen and triptans. The initial treatment was not effective, and the headache persisted. Magnetic resonance (MR) imaging and MR angiography performed 2 days after admission revealed an intramural haematoma of the left vertebral artery, and conventional angiography performed the next day confirmed the presence of the left VAD (Fig. 1). The patient was cautiously treated with anticoagulants and short-term preventive medication for CH (prednisolone for 3 weeks). Her symptom disappeared 10 days after admission. She had no attacks during the follow-up period of 10 months.
Magnetic resonance (MR) images obtained 11 days after the onset of a cluster-like headache (a–c). T1- and T2-weighted MR images at the level of the skull base show subacute stage of intramural haematoma in the intracranial part of the left vertebral artery (white arrows, a and b). On diffusion-weighted images (DWI), there were no signal changes in the medulla and cerebellum (c). Left vertebral artery angiography shows the segmental and diffuse luminal narrowing of the left vertebral artery just below the posterior inferior cerebellar artery (black arrow, d).
Discussion
Our patient had a VAD which presented with recurrent ipsilesional periorbital headache in association with autonomic symptoms. The clinical features of her headache were mostly compatible with the IHS criteria for CH. Our patient had some warning signs and/or atypical features, such as initially developed posterior neck pain, which suggest an underlying causative lesion. In addition to posterior neck pain, we thought that our patient was a candidate for neuroimaging because of her poor response to conventional treatment and the fact that her headache newly developed in her middle age.
In general, the headache of a VAD is distributed over the posterior neck or occipital region due to stimulation of the vertebral artery (7). Anterior or facial pain is rare in VADs without ischaemia (5). Lateral medullary or cervical cord infarctions secondary to VADs may present as a cluster-like headache (4–6). Lesions of the lateral medulla where the descending root of the trigeminal nerve, sympathetic tract and parasympathetic nuclei such as the superior salivatory nucleus are in close proximity, are a reasonable explanation for the development of a cluster-like headache as well as lesions of the cavernous sinus (5). Since our case had no lesions in the lateral medulla, a VAD might have been the only contributing factor to the development of the cluster-like headache. As reported in a previous report, VADs may irritate the cervical innervations over these structures and therefore affect the trigeminal nucleus (6). In the case of Galende et al., a TAC also preceded the development of a lateral medullary infarction, which suggested that perfusion changes in the posterior inferior cerebellar artery territory may play a role in the development of TACs (5). Our case which had a VAD without lateral medullary infarction supports such a hypothesis. Transient ischaemia or perfusion deficit of the lateral medulla may induce trigeminal activation, sympathetic dysfunction and parasympathetic hyperactivity or imbalance between both autonomic systems (5).
In summary, this is an unusual case of spontaneous VAD mimicking CH. Including our case, cluster-like headache may occur in various organic brain lesions that cause structural and/or functional change to anatomical structures involved in trigeminal autonomic pathways. Neuroimaging studies should not be delayed in the presence of the possibility of secondary CH.