The prevalence of headache in Behçet's syndrome.

Abstract

Objectives. Behcet's syndrome is an uncommon systemic disorder that involves the nervous system in 5% of cases. Headache may arise in conjunction with such complications but also appears to occur independently. We sought to define the prevalence of headache in an unselected group of patients with Behcet's syndrome, to characterize the headache syndromes and to identify what treatments are being used. Methods. A questionnaire was sent to an unselected group of patients through their support group newsletter. Results. The results showed a prevalence of recurrent headache in 82.5% of responders; the majority exhibited symptoms that fulfilled the International Headache Society criteria for migraine, with a higher than normal prevalence of visual or sensory aura of 52%. Using the Migraine Disability Assessment (MIDAS) score for disability in migraine, 62% of responders showed moderate or severe disability. Headache treatment was poor, the majority of sufferers resorting to over-the-counter remedies; preventative treatments had rarely been prescribed. Conclusions. Recurrent headache is very common in Behcet's syndrome, is poorly treated and is associated with disablement. Behcet's syndrome is a multisystem disorder of unknown aetiology in which orogenital ulceration, skin lesions and uveitis are common. The disorder may also affect the joints, gut, lungs and the nervous system. For the most part the pathophysiology of the disorder has a vasculitic basis, although this is not seen within the nervous system. Neurological complications involve an inflammatory infiltration or arise as a manifestation of vascular complications (1). Neurological involvement is seen in around 5% of all cases of Behcet's syndrome (2), inflammatory and parenchymal involvement being more common than vascular complications. Headache is a frequent accompaniment of meningoencephalitis and meningitis, in which an escalating, often throbbing, headache develops and worsens over several days. Photophobia, neck stiffness and focal neurological signs often, but not always, follow, and as the syndrome resolves the headache disappears. However, many patients find that headache is a frequent and troublesome symptom in Behcet's syndrome, including those with no clinical evidence for neurological involvement. MRI studies of patients with Behcet's syndrome have shown that in patients with headache as the sole neurological symptom, around half had normal MRI scans and the remainder showed small white-matter lesions within the hemispheres (3, 4). This implies that not all patients with headaches who have Behcet's syndrome also have neuro- logical involvement by means of perivascular inflammatory cell infiltration or by venous occlusion. Another study (5) of 20 patients without neurological signs (the symptom of headache was not noted in the paper) who underwent non-activated SPECT scanning showed decreased or asymmetric HMPAO uptake or both in 35% of cases. Hence there may be a proportion of patients without neurological involvement who may have abnormal cerebral blood flow; whether or not this may be associated with headache or inflammatory neurological disorders is not clear. The purpose of this study was to define the prevalence of headache in an unselected group of patients with Behcet's syndrome, to characterize the headache syndromes and to identify what treatments are being used. Methods