Central nervous system vasculitis in Behçet's syndrome: Angiographic improvement after therapy with cytotoxic agents

Abstract

We describe a patient with Behcet's syndrome who had clinical and angiographic features of widespread cerebral angiitis of small-and medium-caliber vessels. We report the successful treatment of this condition with a combination of cytotoxic agents and steroids, and demonstrate angiographically the resolution of these abnormalities in neuro-Behget's disease.The triad of recurrent aphthous oral and genital ulcers and uveitis was first described by Behqet in 1937 (I). Since then, BehCet's syndrome has been recognized as a multisystem disease that includes neurologic, vascular, articular, and intestinal involvement in addition to the previously described triad (2). Central nervous system (CNS) involvement is a serious complication in Behqet's syndrome, and it most frequently presents as a meningoencephalomyelitic syndrome. Occasionally, it presents as a brain stem syndrome or confusional syndrome (3). Vascular lesions of venous occlusions or aneurysms (4) are characteristically present.We report here a case of Behset's syndrome with clinical and angiographic evidence of CNS vas- culitis. After aggressive therapy with cytotoxic agents and steroids, the patient's condition improved.Case report. The patient, a 39-year-old black woman, first presented to The Cleveland Clinic Foundation in August 1983, with symptoms of pain, swelling, and warmth in both knees. She also described painful "red lumps" on her legs. The lumps were present for days or weeks and then resolved, but left scarring. Her other symptoms included generalized fatigue, a sore tongue, and scotomas of her right eye. Her medical history included mild hypertension of several years duration.Physical examination revealed a 2 x 2-cm tender nodule on the right anterior tibia and multiple areas of hyperpigmentation on the front of her lower legs. At that time, the Westergren erythrocyte sedimentation rate (ESR) was 32 mm/hour. Antinuclear antibody was present at a titer of 1:20, and rheumatoid factor was negative. It was believed that she had postinfectious erythema nodosum. She was given no specific treatment, and the condition subsequently improved.In May 1984, the patient developed acute blindness of her right eye. She was examined at another hospital, and a diagnosis of retinal artery occlusion and retinal detachment was made. She was started on a regimen of prednisone, 20 mglday, for suspected retinal vasculitis. Subsequently, she developed severe cephalgia in the frontal and left temporal areas, but there were no associated visual or neurologic disturbances. A lumbar puncture was performed, and analysis of the cerebrospinal fluid revealed a protein content of 23 mg%; no red blood cells or white blood cells were observed. Computed axial tomography of the patient's head and kidneys showed normal findings, as did renal angiography . Four-vessel cerebral

Neuro-Behçet Disease

“…Arterial involvement can present as stenosis, 83 aneurysm formation, 84 both stenosis and aneurysm formation, 85 or dissection 86,87 of the cerebral arteries. These pathologies cause intracerebral and/or subarachnoid hemorrhage 84,88,89 or arterial infarct.…”

“…These pathologies cause intracerebral and/or subarachnoid hemorrhage 84,88,89 or arterial infarct. 35 The sites of involvement include the common carotid, 90 internal carotid, 87,89 middle cerebral, 84,85 anterior cerebral, 85 anterior communicating, 88 or vertebral 86 arteries. Vasculitis of the vasa vasorum is suspected as the basis of aneurysm formation or arterial dissection.…”

“…30 Neurovasculo-Behçet disease must be confirmed by neuroimaging procedures like CT angiography, magnetic resonance angiography, MRI, or conventional angiography. 30,79,85,89 …”

Cerebrovascular manifestations of Behçet’s disease

“…Intracranial cerebral artery stenosis or occlusion is very rare, and only two cases have been reported in the literature to date. [18][19][20] The constellation of cerebral vasculitis and brain infarction represents a particular and unusual form of neuro-Behçet's syndrome. Ischemic stroke and cerebral vasculitis are unusual manifestations during the course of BD, and only two cases have been reported in the literature so far.…”

Behçet's Disease-Associated Aneurysm of the Internal Carotid Artery within the Cavernous Sinus: Report of a Patient

“…There are reports of the successful treatment of unruptured aneurysms with endovascular modality and immunosuppressive drugs including cyclophosphamide and steroids. 17,20 However, immunosuppressive treatment may not always be successful. 12 In our patient, the aneurysm size increased threefold in one year despite cyclophosphamide and steroid therapy and thus necessitated endovascular treatment.…”