Recurrent Stroke as a First Presentation in Behçet Disease: A Case Report

Behçet disease (BD) is a multisystemic relapsing autoimmune vascular disorder. It is clinically characterized by recurrent oral ulcers, genital ulcers, eye, and skin manifestations. Development of neurological symptoms in BD cases is rare and occurs several years after the initial diagnosis. We desc...

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Published inCurēus (Palo Alto, CA) Vol. 15; no. 11; p. e49222
Main Authors Khafaji, Salah, Alzahrani, Meshari S, Muddassir, Rabia, Almuhanna, Rakan A
Format Journal Article
LanguageEnglish
Published United States Cureus Inc 22.11.2023
Subjects
Aneurysms
Antibodies
Case reports
Diabetes
Dissection
Emergency medical care
Hemorrhage
Ischemia
Magnetic resonance imaging
Medical imaging
Monoclonal antibodies
Mutation
Stroke
Tomography
Ulcers
Vein & artery diseases
neuro-behçet disease
brainstem lesion
vasculitis
recurrent stroke in young
behcet disease
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Summary:Behçet disease (BD) is a multisystemic relapsing autoimmune vascular disorder. It is clinically characterized by recurrent oral ulcers, genital ulcers, eye, and skin manifestations. Development of neurological symptoms in BD cases is rare and occurs several years after the initial diagnosis. We describe a rare case of a 39-year-old Saudi male who presented with isolated neurological manifestations as the first sign of BD. The patient had recurrent strokes, both ischemic and hemorrhagic, over an 11-month period before developing typical BD features. A thorough investigation excluded other potential etiologies of his neurological disorders. Imaging showed multiple brainstem lesions compatible with parenchymal neuro-BD (NBD). The patient was positive for HLA-B51, a genetic marker linked to BD, but had a negative pathergy test. Treatment with corticosteroids and infliximab resulted in symptom improvement. The diagnosis of NBD requires a comprehensive clinical, imaging, and laboratory assessment to rule out other possible causes. This case demonstrates the need to include NBD in the differential diagnosis of young patients with unexplained neurological manifestations, especially if they are followed by an onset of BD features. Treatment with corticosteroids and biologic agents can achieve favorable outcomes. NBD can present with isolated neurological symptoms, emphasizing the need for a high level of suspicion and a multidisciplinary approach for accurate diagnosis and effective management.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.49222