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Recurrent orogenital ulcers with papilloedema and headaches

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Q1: What is the initial diagnosis?

Behçet's syndrome.

Q2: What does the unenhanced computed tomogram of the brain show (see p 54)?

This is a normal unenhanced computed tomogram of the brain.

Q3: What probable complication of the initial diagnosis has occurred?

Dural sinus thrombosis.

Q4: What further investigation will you do to confirm the complication?

Magnetic resonance imaging venogram or computed tomogram of the brain with contrast.

Q5: What is the treatment of this complication?

Anticoagulation with heparin and warfarin.

Discussion

Behçet's syndrome is a chronic relapsing occlusive vasculitis affecting multiple organ systems in the body. The international study group for Behçet's disease proposed the following diagnostic criteria.1

Recurrent oral ulcers plus two of the following:

  • Recurrent genital ulcers.

  • Ocular involvement.

  • Skin lesions.

  • Positive pathergy test.

Oral involvement is usually in the form of recurrent aphthous ulcers that heal in several days without leaving any scar.2,3

Genital ulcers occur on the scrotum in males and on the labia in female patients and heal with scarring.4 Skin manifestations are usually in the form of erythema nodosum, acneiform lesions, or pseudofolliculitis on the face and back of patients, and migratory thrombophlebitis.5 Recurrent anterior uveitis with hypopyon formation and, less frequently, retinal vasculitis are the main manifestations in the ocular involvement of Behçet's disease.2,3 Other ocular presentations include episcleritis, scleritis, retinal vein thrombosis, and corneal and conjunctival ulcers.6 Cataract formation is the most common anterior segment complication after recurrent inflammation, occurring in about 36% of patients.7 Other systems can be involved, primarily due to the thrombotic and vaso-occlusive changes affecting vessels of all sizes, as in our case where the venous drainage of the brain was affected causing dural sinus thrombosis.

The skin pathergy reaction is a non-specific, hyper-reactive lesion (papulopustular) at the site of trauma or a needle prick. When present the reaction is specific for Behçet's disease.8

Dural sinus thrombosis may be acute or chronic and may present with non-specific symptoms, such as headache, nausea and vomiting, altered mental status, and seizures. Focal neurological complaints may also be present. The clinical differential diagnosis for dural sinus thrombosis includes cerebrovascular accident, intracranial neoplasm, vascular malformation, and benign intracranial hypertension. Magnetic resonance imaging (MRI; fig 1) and MRI venogram9 or computed tomography of the brain with contrast are valuable in diagnosing dural sinus thrombosis. Unenhanced computed tomography of the brain is less sensitive in delineating a sinus thrombosis and may appear falsely negative.10 Treatment of dural sinus thrombosis is usually anticoagulation with heparin11 and warfarin. Local infusion of urokinase, a thrombolytic agent, is being tried and holds promise.12

Figure 1

Sagittal MRI scan showing venous sinus thrombosis in the superior sagittal sinus.

Final diagnosis

Behçets syndrome with dural sinus thrombosis as a complication.

References

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