Background: Behcet disease (BD) is a rare small vessel vasculitis that commonly manifests as recurrent painful oral or genital ulcerations, uveitis, and skin lesions. Some patients with BD develop neurological symptoms termed neuro-Behcet's disease. In the emergency department setting, these symptoms can be mistaken for other common acute issues including stroke, infection, epilepsy, multiple sclerosis, toxin ingestion, or psychiatric conditions.
Case Report: We present a case of a 28-year-old male with neuro-BD mimicking status epilepticus and meningoencephalitis. He was actively seizing on arrival and febrile at 103.8 F. The patient also had a history of vasculitis, uveitis, and genital lesions which raised suspicion for a rheumatological process. Cerebrospinal fluid samples were obtained and computed tomography of the head revealed no acute findings. The patient was started on treatment for seizure and meningoencephalitis and admitted to neurology for continuous electroencephalogram monitoring with additional consults placed for rheumatology, ophthalmology, and infectious disease. The patient was ultimately diagnosed with neuro-Behcet's disease and treated appropriately. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: In a patient presenting with neurological symptoms and signs of rheumatological disease providers should consider neuro-Behcet's disease as a differential diagnosis. This case exemplifies the vital role of reviewing past medical history to expand differential diagnoses and early coordination with specialists so treatment can be initiated early to prevent morbidity and mortality. The case will also review different presentations of this unfamiliar diagnosis for a more comprehensive understanding.
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http://dx.doi.org/10.1016/j.jemermed.2024.09.024 | DOI Listing |
J Emerg Med
October 2024
Department of Emergency Medicine, Cleveland Clinic Akron General, Akron, Ohio; Northeast Ohio Medical University, Rootstown, Ohio. Electronic address:
Background: Behcet disease (BD) is a rare small vessel vasculitis that commonly manifests as recurrent painful oral or genital ulcerations, uveitis, and skin lesions. Some patients with BD develop neurological symptoms termed neuro-Behcet's disease. In the emergency department setting, these symptoms can be mistaken for other common acute issues including stroke, infection, epilepsy, multiple sclerosis, toxin ingestion, or psychiatric conditions.
View Article and Find Full Text PDFClin Exp Immunol
January 2025
Department of Clinical Laboratory, State key Laboratory of Complex, Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, Beijing, China.
Neuro-Behçet's disease (NBD) is a more severe but rare symptom of Behçet's disease (BD), which is mainly divided into parenchymal NBD (p-NBD) involving brain stem, spinal cord, and cerebral cortex. Non-p-NBD manifests as intracranial aneurysm, cerebral venous thrombosis, peripheral nervous system injuries, and mixed parenchymal and non-parenchymal disease. P-NBD is pathologically characterized by perivasculitis presenting with cerebrospinal fluid (CSF) pleocytosis, elevated total protein, and central nervous system (CNS) infiltration of macrophages and neutrophils, which are subdivided into acute and chronic progressive stages according to relapsing-remitting courses and responses to steroids.
View Article and Find Full Text PDFZhonghua Er Ke Za Zhi
January 2025
Department of Rheumatology and Immunology, Children's Hospital Affiliated to Capital Institute of Pediatrics, Beijing100020, China.
Clin Case Rep
December 2024
Multiple Sclerosis Research Center, Neuroscience Institute Tehran University of Medical Sciences Tehran Iran.
This case highlights the importance of considering Neuro-Behçet's disease (NBD) in the differential diagnosis of multiple sclerosis (MS), particularly in patients presenting with neurological manifestations, abnormal magnetic resonance imaging (MRI) findings, and systemic symptoms consistent with Behçet's disease (BD).
View Article and Find Full Text PDFRadiol Case Rep
January 2025
Central Radiology Department, Ibn Sina University Hospital Center, Mohamed V University of Rabat, Rabat, Morocco.
Neuro-Behçet disease is often difficult to diagnose due to its complex and severe clinical presentation. This article reports the case of a 35-year-old female patient with a history of Behçet's disease, admitted for a deep coma. Brain MRI performed upon admission revealed extended lesions in the basal ganglia, thalami, and midbrain, along with leptomeningeal contrast enhancement in the temporal region, suggesting meningoencephalitis compatible with parenchymal involvement of neuro-Behçet's disease.
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