Behcet's disease presenting with cerebral vasculitis: a case report
© licensee BioMed Central Ltd. 2009
Received: 11 September 2008
Accepted: 29 April 2009
Published: 26 May 2009
Behcet's disease encompasses a group of multisystemic complications secondary to occlusive vasculitis. It presents usually with oral or genital ulcers however, other system involvements may be the first sign as well.
A 26-years-old man admitted to our clinic for his decreased visual acuity. Ophthalmologic examination revealed bilateral vitritis and optic disc swelling. However, the meticulously taken history included other complains like headache and oral ulcers. Further investigation with cranial MRI demonstrated cerebral vasculitis secondary to Behcet's disease.
Behcet's disease may be easy to diagnose when it presents with oral and genital ulcers or panuveitis, however presentation with optic disc swelling may warrant a thorough investigation.
Behçet's Disease (BD) is a chronic, multisystemic, inflammatory disorder characterized by intraocular inflammation, oral and mucosal ulcerations, skin lesions, and a variety of other disorders . This disease with distinctive prevalence along the Silk Route and between the ages of 20-35 years is of utmost importance for the ophthalmologists as 50-80% of cases presented ocular involvement. The chronic, recurrent uveitis affecting the anterior and posterior uveal tract usually bilateral is the major cause of morbidity. The posterior segment involvement is described as retinal vasculitis and accompanied generally by vitritis and macular edema .
Though optic disc edema is a frequent finding in BD, differential diagnosis between papillitis and papilledema is warranted and cases without uveitis should be suspected inevitably for central nervous system (CNS) involvement . A young male diagnosed to have BD with both ocular and central nervous system involvements as the initial finding is presented herein.
BD is a chronic, relapsing, occlusive vasculitis of unknown etiology, affecting almost every organ system in the body. Though involvement of CNS is less common compared to ocular involvement, it may lead to lethal complications. Its prevalence is reported to be 2.2-4.9%, rising up to 5-10% in larger series and to 20% in autopsy series [3, 5, 6]. On the other hand, the incidence of initial involvement at the time of diagnosis is 5% for CNS and 20% for the eye according to various studies . Ocular findings are frequently seen within the first years after diagnosis, while neurological findings are noticed 4-6 years after diagnosis, but the male predominance is noticed in both organ involvements [2, 6].
Differential Diagnosis of Optic Disc Swelling
Pain with eye movements
Relative afferent pupil defect
May be in posterior vitreus
Optic disc cupping
Hemorrhages and exudates around the optic disc
Enlarged blind spot
CNS involvement may be established in two different patterns in Behcet's disease: parenchymal and non-parenchymal. Parenchymal involvement is the most common form (82%) with a tendency to produce focal lesions clustering especially in the brainstem while the non-parenchymal type mainly result from vascular involvement and presents as dural sinus thrombosis, aseptic meningitis and arterial vasculitis. This type of manifestation is also called as vasculo-Behcet's disease and has a better prognosis [1, 3]. Cerebral vasculitis is a rare type of non-parenchymal CNS involvement and MRI is reported to be highly sensitive and faithful imaging modality for diagnosis .
Behcet's disease is characterized by occlusive vasculitis however presentation with vitritis and papilledema as the initial findings is not frequent . On the other hand, differential diagnosis of cerebral vasculitis did not include Behcet's disease as the most frequent cause. Therefore taking the history of patients meticulously, especially in countries on the Silk Road, which are supposed to have a higher incidence of Behcet's disease, will prevent to overlook the supportive findings like uveitis, oral and genital ulcerations and enable to enlighten the dilemma rapidly and correctly.
Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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