Case Presentation: 55-year-old male with past medical history of diabetes mellitus type 2, complete heart block status post pacemaker, nonischemic cardiomyopathy and Crohn’s disease was admitted for headache, loss of consciousness and seizures. Physical exam showed nuchal rigidity. Complete blood count showed mild leukocytosis. Urinalysis was benign and x-ray chest didn’t show any infiltrates. CT head did not show any structural abnormalities. Urine drug screen was negative. Blood cultures were taken and patient was started on acyclovir, vancomycin and ceftriaxone. CSF analysis showed total cell count 90/ul, CSF glucose 126mg/dl, protein 73mg/dl with 30% PMNs and 47% lymphocytes. EEG revealed no evidence of seizure. MRI brain could not be completed given pacemaker incompatibility. CSF culture revealed no growth. HSV, CMV and VZV PCR was negative. Anti-NMDA receptor antibodies in CSF were not detected. CSF VDRL was nonreactive and EBV and West Nile serologies were negative. HIV and autoimmune panel was negative. ESR, CRP were significantly elevated. Review of records revealed prior episode of meningitis 7 years ago when patient was admitted with similar symptoms and was diagnosed as having aseptic meningitis. CSF analysis at that time showed total cell count 63/ul, CSF glucose 84mg/dl, protein 115mg/dl with 72% lymphocytes and 11% PMNs with negative blood and CSF cultures. Blood and CSF cultures remained negative throughout hospitalization and antibiotics and acyclovir was discontinued. Patient was started on high dose intravenous methylprednisone with dramatic improvement in mentation. Patient gave history of at least four prior episodes of aseptic meningitis and reported recurrent facial and scalp pustules and painful oral ulcers with spontaneous resolution. There was no established diagnosis of Behcet’s disease in the past. Patient reported a prior episode of eye redness, pain and photophobia and ongoing knees and shoulder arthritis however denied any genital ulcers. Patient also reported similar symptoms in his father. Patient was eventually discharged home with tapered dose of steroids.
Discussion: Behcet’s disease (BD) is an uncommon systemic vasculitis characterized by relapsing episodes of multisystem inflammation with unclear etiology. Signs and symptoms include oral and/or genital ulcers, skin and ocular lesions, arthritis, gastrointestinal ulceration and vascular thrombosis. Diagnosis is mainly clinical and disease management depends on extent and severity of affected organ. Neuro-Behcets’s disease (NBD) can present with a variety of neurological symptoms i-e multiple sclerosis, brainstem and meningoencephalitic syndromes. Our patient presented with multiple episodes of aseptic meningitis with CSF analysis showing neutrophilic pleocytosis with negative gram stain and cultures consistent with neurological involvement. He met criteria for diagnosis with mucocutaneous (skin and oral lesions), ocular, joint, cardiac (nonischemic cardiomyopathy, heart block) and gastrointestinal involvement (inflammatory bowel disease). Corticosteroids and immunosuppressive medications are mainstay of treatment.
Conclusions: NBD should be considered in the differential diagnosis of patients presenting with signs and symptoms of meningitis. Awareness of clinical manifestations of BD is key for timely and accurate diagnosis of NBD as early treatment with corticosteroids and other immunosuppressive medications can prevent long term mortality and morbidity.