Clinical and cytokine profile of adult acute necrotizing encephalopathy

• Published in: Biomedical Journal Vol. 42; no. 3; pp. 178 - 186
• Main Authors: Lin, Yi-Ying, Lee, Kuang-Yung, Ro, Long-Sun, Lo, Yen-Shi, Huang, Chin-Chang, Chang, Kuo-Hsuan
• Format: Journal Article
• Language: English
• Published: United States Elsevier Limited 01.06.2019; Chang Gung University; Elsevier
• Subjects: Acute Disease; Adult; Aged, 80 and over; Antigens; Brain - metabolism; Brain - pathology; Brain Diseases - blood; Brain Diseases - drug therapy; Brain Diseases - pathology; Cell adhesion & migration; Cerebrospinal fluid; Children; Consciousness; Cornea; Cytokines; Cytokines - blood; Diarrhea; Encephalopathy; Female; Fever; Growth factors; Humans; Inflammation; Interleukin 6; Laboratories; Magnetic Resonance Imaging - methods; Male; Molecular Chaperones - blood; Neuroimaging; NMR; Nuclear magnetic resonance; Original; Patients; Pediatrics; Posture; Proteins; Rare diseases; Serum levels; Signs and symptoms; Transforming Growth Factor beta1 - blood; Transforming growth factor-b1; Tumor necrosis factor-TNF; Vascular cell adhesion molecule 1; Viral infections; Adult; VCAM-1; Acute necrotizing encephalopathy; Cytokine
• ISSN: 2319-4170; 2320-2890; 2320-2890
• DOI: 10.1016/j.bj.2019.01.008

Acute necrotizing encephalopathy (ANE), a fulminant encephalopathy, is often found in childhood. It is still uncertain whether adult patients with ANE display clinical features different from patients with typical pediatric onset. Furthermore, alterations in neuroinflammatory factors in patients with ANE have not been well-characterized. Here, we present an adult patient with ANE, and review all reported adult ANE cases in the literature. Serum levels of five cytokines were checked in an adult patient with ANE and compared with gender/age-matched controls. Literature search was performed with PubMed, using the term as "acute necrotizing encephalopathy" with the filter of adult 19 + years. A total of 13 adult patients were reviewed. Compared with pediatric patients, adult ANE patients had similar clinical symptoms, biochemical data, and neuroimage findings, whereas adult ANE were more female-biased (female:male, 9:4) with a worse prognosis. Elevated cytokine levels in the serum and/or CSF is found in both adult-onset and pediatric-onset ANE. We found significantly elevated serum levels of IL-6 (17.17 pg/mL; healthy control: 1.43 ± 1.22 pg/mL) and VCAM-1 (3033.92 ng/mL; healthy control: 589.71 ± 133.13 ng/mL), and decreased serum TGF-β1 level (14.78 ng/mL, healthy controls: 25.81 ± 6.97 ng/mL) in our patient. Our findings clearly delineate the clinical features and further indicate the potential change in cytokine levels in adult patients with ANE, advancing our understanding of this rare disease.