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Behçet’s disease (BD) is a multisystemic recurrent inflammatory disorder, which is originally described as a triad of oral and genital ulcerations with uveitis. As vasculitis of the vasa vasorum is the main pathological hallmark of BD, it is generally seen in the form of superficial thrombophlebitis or occlusion of major veins; however arterial obstruction and aneurysms may also be seen to a lesser extent.1 We present a patient with BD who developed bilateral internal carotid artery (ICA) occlusions.
A 43 year old, right handed male patient was referred to Ege University Neurology Department for evaluation of an acute onset right sided weakness, fever, headache, and difficulty with gait and speech in August 2001.
On admission, he was alert and fully oriented. His temperature was 38°C, pulse was regular (90/min), blood pressure was 150/80 mm Hg. His speech was severely dysarthric but he could name, repeat, read, and follow instructions. His cranial nerves and fundoscopic examination were normal. His gait was wide based and unsteady. He had four sided mild weakness, which was prominent on the right. Muscle stretch reflexes were normal but plantar reflexes were extensor bilaterally. His coordination was impaired in proportion to weakness in all four extremities. He had mild nuchal rigidity of the neck with positive Brudzinski’s sign. On physical examination, erythema nodosum like dark red, painful lesions were noticed on both anterior aspects of the legs. His ophthalmological examination did not reveal any signs of uveitis. He also complained of pain and fever in his scrotum, and urological examination showed swelling, induration, and marked tenderness of epididymia on both sides as the clinical findings of epididymitis.
His medical history showed that he had complained about recurrent oral aphthous lesions and aforementioned skin lesions for 8 to 10 years without medical consultation. He had no other medical history associated with BD. He was a moderate cigarette smoker for 20 years.
Laboratory tests were consistent with an inflammatory condition with a high erythrocyte sedimentation rate (100 mm 1st h) and C reactive protein (12.27 mg/dl; normal range 0–5 mg/dl) levels. CSF examination, serum immunoglobulin levels, platelet count, protein C, protein S, antithrombin III, C3 and C4 complement, rheumatoid factor, and lipid levels were within the normal range. Serum antinuclear, antineutrophil cytoplasmic and anticardiolipin antibodies were negative. ECG, 2D echo, chest radiograph, abdominal ultrasonography, and colour Doppler ultrasonography of the lower extremity vessels were normal. Cranial magnetic resonance imaging showed diffuse cerebral atrophy and chronic ischaemic lesions in both cerebral hemispheres as well as the absence of the flow void in both ICAs on T2 weighted axial images. Digital substraction angiography (DSA) showed complete occlusion of the bilateral internal carotid arteries just rostral to the bifurcation (fig 1).
After consultation with the rheumatology clinic, a pathergy test was performed to confirm the diagnosis of BD and found to be positive. The patient was then transferred to the rheumatology clinic. He was treated with aspirin 300 mg/day, prednisolon 1 mg/kg/day, pentoxifylline 1200 mg/ day, 750 mg pulse cyclophosphamide monthly for BD. He was also treated with oral antibiotics and analgesics for the epididymitis. Two months later, he had almost completely recovered.
Our patient had presented with unusual neurological findings for a classic stroke syndrome and MRI showed bihemispheric ischaemic lesions and bilateral ICA occlusion, which was also shown by DSA. It is known that cardiovascular risk factors, smoking, fibromuscular dysplasia, or moyamoya disease are frequently found as an aetiological factor in patients with bilateral ICA occlusion, whereas essential thrombocytaemia, giant cell arteritis, and BD are among the very rare causes.2,3
Although our patient did not have cardiovascular risk factors except for smoking, he had been suffering from BD for about 10 years, which was not diagnosed before neurological presentation. His medical history, skin lesions, and urogenital findings supported with a positive pathergy test verified the diagnosis of BD according to latest diagnostic criteria for BD.4
Neurological involvement in BD has been reported to occur in 2.2% to 43% of cases in large series, either in the form of neuro-Behçet disease (parenchymal CNS involvement) or vasculo-Behçet disease (secondary or non-parenchymal CNS involvement) or both.1,5 Neuro-Behçet’s disease has a characteristic clinical picture with male predominance and typical cranial MRI findings of reversible inflammatory parenchymal lesions, attributable to small vessel disease, which may rarely be confused with those of MS.5 On the other hand, vasculo-Behçet’s disease is attributable to large vessel disease generally in the form of cerebral venous thrombosis and has limited symptoms with a better prognosis.1,5 Our patient’s neurological signs and symptoms were highly suggestive of neuro-Behçet; however CSF findings with acellularity and normal protein level and neuro-imaging studies showing ischaemic lesions and bilateral ICA occlusions supported a very unusual type of vasculo-Behçet.
Diffuse cerebral atrophy and survival with minimal or no neurological deficit as in our patient is not infrequent in patients with bilateral ICA occlusion. This is explained by the adequate collateral flow provided by vertebrobasilar system and slow, gradual occlusion.2
Occlusive lesions in the bilateral ICAs, as seen in our patient, are extremely rare in BD and we suggest that this is a very unusual case of vasculo-neuro-Behçet’s disease. We also conclude that BD should always be remembered as an aetiological factor for bilateral ICA occlusions, especially in countries where the disease is highly prevalent.