¹Syeda Rida E Zehra, ¹Tahira Perveen, ¹Lubna Nazir
¹Liaquat National Hospital and Medical College

Purpose Statement: Introduction

Behcet’s disease is a chronic, multisystem inflammatory disorder characterized by recurrent oro-genital ulceration, ophthalmologic, nervous, or dermatologic manifestation. There is no pathognomic laboratory test available therefore the diagnosis can be challenging, especially in patients with limited clinical features. Nervous system involvement termed as Neuro-Behcet’s is a relatively rare occurrence. It is sub-classified into two major forms i.e. parenchymal and non-parenchymal. Here we describe a case of Neuro-Behcet’s in a young women with a rather bizarre presentation.

Method(s): A 38 years old female with a history of Behcet’s disease diagnosis 3 years ago presented to the Emergency Department with the complaint of altered sensorium for 3 days. This was preceded by bilateral eye redness for 7 days, and low grade fever for 5 days. Physical examination was notable for an irritable young woman with a GCS of E3V3M5 with no focal deficit on neurological exam. Ophthalmologic examination was consistent with anterior uveitis. MRI brain was performed which demonstrated multiple T2 high signal intensity areas in cortical and subcortical regions of bilateral frontal, parietal, occipital, temporal lobes, left caudate and lentiform nuclei, and both cerebellar hemispheres. Majority high signal on T1. MRA and MRV showed normal vessels. Lumber puncture was performed and CSF DR revealed increased protein content; CS, Viral PCR, and MTB PCR all negative.

She was diagnosed as a case of Behcet’s disease 3 years ago when she had presented to Rheumatology clinic with a cold left foot associated with ankle swelling. CT angiogram left leg at that time was consistent with arterial thrombosis, demonstrating partial mural thrombosis in the distal abdominal aorta, non-opacification of  left superficial femoral artery and partial thrombosis of left popliteal artery. 10 years prior to this presentation she had an episode of Pan-uveitis which was treated with oral corticosteroids. Laboratory tests were significant for a positive fluorescent ANA, negative dsDNA, ENA profile, APS antibodies, and negative ANCAs. She was advised Methotrexate and corticosteroids, but discontinued follow-up 2 years prior to the current presentation to ED.

We started her on IV methylprenisolone 500mg/day for 5 days. 2 days later, patient showed remarkable improvement in irritability and GCS improved to E4V4M6. She was discharged on oral corticosteroid and Methotrexate.

Result(s): Neuro-Behcet’s disease is a rare disease which may leave devastating impact on neurological health. Timely recognition and prompt treatment warrants remission.

Conclusion(s): This case report underscores the complexity and variability of Neuro-Behcet’s disease presentation. It signifies early recognition and prompt intervention to prevent potentially devastating consequences of this rare disease. Most of the literature on Neuro-Behcet’s disease in the world comprises personal experiences and reports; our case is a contribution to this growing body of literature to enhance better understanding of the disease.