Abstract/ Resumo
Behcet's disease (BD) is a multisystem inflammatory disorder characterized by recurrent oral and genital
ulcers, skin lesions and
uveitis. The nervous system involvement of BD, neuro-
Behcet's disease (NBD), is one of the important causes of mortality of the disease. Herein, we present a 29-year-old male with parenchymal NBD who has progressed rapidly and was managed with an uncommon aggressive immunosuppresive combination
therapy. The patient first presented six years ago with
vertigo and difficulty in talking and walking. On examination, he had
oral ulcers, acneiform lesions on the torso, genital
ulcer scar, dysartria, and
ataxia. Along with the magnetic resonance imaging (MRI) findings, the patient was diagnosed as NBD. After pulse
methylprednisolone (1g/day, 3 days) and 8 courses of 1g/month iv cylophosphamide
therapy, he was put on
azathioprine and oral methlyprednisolone. On the 4th year of the maintenance
therapy, he was admitted with NBD relapse which was treated with 3 days of iv 1g pulse methlyprednisolone. One year after the last relapse, the patient voluntarily stopped medications and presented with
global aphasia, right hemihypoesthesia and
quadriparesis. MRI findings were suggestive of NBD relapse. After exclusion of
infection, pulse
methylprednisolone was started but no improvement was observed. Considering the severity of the NBD, the patient was put on
methylprednisolone (1mg/kg/day), iv cylophosphamide (1g) and
adalimumab 40 mg/14 days subcutaneously with appropriate
tuberculosis prophylaxis. Neurological examination and MRI findings after 4 weeks showed dramatic improvement however patient developed
pulmonary tuberculosis.
Methylprednisolone dose was decreased (0.5mg/kg/day) and quadruple antituberculosis
therapy was started. Patient was discharged with 5/5 muscle strength in extremities without any respiratory symptoms 2 months after first presentation. Prompt introduction of immunosuppressive therapy is crucial in NBD. Although combination of
TNF inhibitors and cyclophoshamide is a rare therapeutic approach, it may be life-saving. However a higher awareness is required for
opportunistic infections.