Behçet Disease and Cognitive Impairment: A Case Study of an Overlooked Symptom

Objective: Unusual clinical course Background: Behçet disease (BD) is a rare systemic vasculitis with diverse clinical manifestations, most commonly oral ulcers. Neurological involvement occurs in 5 to 15 of cases, often presenting as brainstem and myelopathic syndromes. However, cognitive impairment can develop even in the absence of overt neurological signs. A case-control study using the Brief Repeatable Battery of Neuropsychological Tests reported cognitive dysfunction in 38 of BD patients lacking overt neurological symptoms, suggesting that the cognitive dysfunction was immune-mediated. This case report points to the importance of heightened clinical vigilance in patients with BD presenting with subtle cognitive changes, even in the absence of neurological involvement. Routine neuropsychological screening could enable earlier detection and timely intervention, thereby reducing the risk of irreversible cognitive decline. Case Report: A 59-year-old woman with BD presented with progressive cognitive impairment 1 year after diagnosis. She had no prior cognitive problems or focal neurological signs. Her comorbid thymoma and hyperthyroidism remained clinically stable. Cognitive screening (MoCA-INA 27/30) revealed deficits in memory and attention, supported by suboptimal performance on the Digit Span and Trail-Making tests. Brain magnetic resonance imaging (MRI) ruled out stroke or neurodegeneration but demonstrated bilateral parietal atrophy and mild white-matter hyperintensities in the temporal and occipital lobes. She received donepezil, folic acid, cognitive stimulation, and immunosuppressive therapy. At 1-year follow-up, the Montreal Cognitive Assessment (MoCA-INA) score had normalized (30/30), with improved neurocognitive performance and stable daily functioning. Conclusions: This case shows that cognitive impairment can present as an isolated manifestation of BD, even in the absence of overt neurological symptoms. Early neuropsychological screening and timely intervention can prevent progression and preserve quality of life. © Am J Case Rep, 2025.