Department of Radiology, Aga Khan University, National Stadium Road, Karachi, 74800, Pakistan.
Department of Pediatrics & Child Health, Aga Khan University, Karachi, Pakistan.
Pediatr Radiol. 2024 Nov;54(12):2026-2035. doi: 10.1007/s00247-024-06058-5. Epub 2024 Sep 28.
Acute necrotizing encephalopathy of childhood is a unique entity with bilateral gray and white matter involvement.
The aim of this study is to explore whether the severity of findings on imaging scans is indicative of the prognosis and clinical outcomes for pediatric patients with acute necrotizing encephalopathy.
A retrospective cross-sectional study was conducted on 42 patients diagnosed with acute necrotizing encephalopathy. A severity score based on MR imaging was computed for each patient, utilizing a point system determined by the existence of factors such as hemorrhage, cavitation, enhancement, diffusion restriction, and lesion location. The scoring was categorized into mild, moderate, and severe. Clinical outcomes were determined at the time of discharge and at follow-ups as mild disability, moderate disability, severe disability, and death according to the modified Rankin Scale. Associations were determined by Fisher's exact test, chi-square test, and one-way ANOVA.
The study included 21 boys and 21 girls with a mean age of 71.5 months. A statistically significant connection (P=0.027) was found between the severity score from MR imaging and the clinical outcome. A statistically significant relationship was also observed between diffusion restriction (P=0.008), cerebellar involvement (P=0.048), and an unfavorable clinical outcome. Additionally, individuals who experienced shock exhibited a correlation with adverse outcomes (P=0.01).
In predicting the outcome of acute necrotizing encephalopathy, cerebellar involvement and presence of diffusion restriction were associated with worse clinical outcomes in our study. Developing a comprehensive MR-based severity score is crucial for improving diagnostic accuracy and patient outcomes. Our findings underscore the importance of including diffusion restriction and cerebellar involvement in the scoring system.
儿童急性坏死性脑病是一种具有双侧灰白质受累的独特实体。
本研究旨在探讨影像学扫描的严重程度是否能反映儿童急性坏死性脑病患者的预后和临床结局。
对 42 例诊断为急性坏死性脑病的患者进行了回顾性横断面研究。为每位患者计算了基于磁共振成像的严重程度评分，采用了一种点系统，该系统由出血、空洞、增强、弥散受限和病变部位等因素的存在来确定。评分分为轻度、中度和重度。临床结局在出院时和随访时根据改良 Rankin 量表确定为轻度残疾、中度残疾、重度残疾和死亡。采用 Fisher 精确检验、卡方检验和单向方差分析确定关联。
本研究纳入了 21 名男孩和 21 名女孩，平均年龄为 71.5 个月。MR 成像的严重程度评分与临床结局之间存在统计学显著关联（P=0.027）。弥散受限（P=0.008）、小脑受累（P=0.048）与不良临床结局之间也存在统计学显著关系。此外，休克患者与不良结局相关（P=0.01）。
在预测急性坏死性脑病的结局时，小脑受累和弥散受限与本研究中更差的临床结局相关。制定全面的基于磁共振的严重程度评分对于提高诊断准确性和患者结局至关重要。我们的研究结果强调了在评分系统中纳入弥散受限和小脑受累的重要性。
