No abstract

Natural killer (NK) cells play a critical role in the host defense against herpesviruses. Although herpesviruses are ubiquitous in human populations, only a minority of people experience severe recurrent infections. We hypothesize that uncharacterized NK cell functional deficits predispose individuals to more significant or frequent herpesvirus infections and reactivations. To investigate this hypothesis, we broadly analyzed NK cell phenotype and functional responses in a cohort of predominantly pediatric patients with recurrent and/or severe herpesvirus infections and compared them to a healthy control population. Our results identified no global differences in cytolysis, degranulation, interferon-γ production, or surface receptor upregulation following cytokine stimulation. However, abnormal NK cell functional responses were observed in nearly one-third of patients (including 3 with hyporesponsiveness to activating signals and 1 with markedly decreased CD11b expression associated with reduced cytotoxicity and degranulation), which might contribute to those individuals' susceptibility to herpesvirus infections.

We describe a case of neuro-Behçet disease diagnosed in a 12-year-old girl. This patient presented with recurrent oral ulcers, incontinence, spastic gait, blurry vision, and asymmetrical lower extremity hypertonia. Extensive testing revealed punctate lesions through the central nervous system, vitritis, papillitis, and uveitis. A thorough infectious and neoplastic workup was negative. She was treated with pulse steroids and azathioprine with gradual improvement in her gait and ophthalmologic findings. Although rare, primary neuro-Behçet should be considered in pediatric patients with neurologic abnormalities and recurrent aphthous ulcers without other explanation.