Unravelling the Inflammatory and Metabolic Insights in Subacute Sclerosing Panencephalitis: A Prospective Multimodal Evaluation

IF 2 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology Pub Date : 2026-01-01 Epub Date: 2025-10-22 DOI:10.1016/j.pediatrneurol.2025.10.012

Prashant Jauhari MD, DM , Sakshi Ojha MD, DM , Madhavi Tripathi MD , Lata Singh PhD , Vishwesh Bharadiya MBBS , Amulya Gupta MBBS , Parul Berry MBBS , Gagneet Kaur MBBS , Atin Kumar MD , Ashish Upadhyay PhD , Sheffali Gulati MD

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Abstract

Background

This study discusses results of multimodal evaluation of biomarkers of neuroinflammation and neurodegeneration in cerebrospinal fluid (CSF) and Fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) findings in Subacute sclerosing panencephalitis (SSPE).

Methods

Consecutive children (3-14 years) with SSPE fulfilling the Dyken criteria during the study period (2020-2022) were enrolled. Clinical profile, radiological and electroencephalography data were chronicled. CSF interleukins (ILs) IL-4, IL-6, and IL-10; interferon-gamma; and neurofilament heavy chain (NFH) levels were analysed and compared with age matched pseudo-controls. 18F-FDG-PET imaging was performed in a subset of children who consented for the same.

Results

Thirty-three children with SSPE were enrolled [mean age 8.06 ± 2.67 years; males: 82%; Jabbour Stage I-6%, Stage II-67%, Stage III-27%]. Two forms of presentation were seen: short/absent stage I with early onset myoclonic jerks (N = 22; mean age of onset 7.85 ± 2.45) and prolonged stage I with late onset epilepsy (N = 11; mean age 6 ± 1.9 years, P = 0.03). CSF NFH was significantly elevated in SSPE [132.62 (53.6-388.9) vs 0.0 (0-16.9) pg/ml; P = 0.0001] compared to the pseudo-controls. CSF IL-6 (pg/ml) in 21/22 SSPE [1.72 (0.85-3.04)] children and 16/17 pseudo-controls [2.60 (1.72-3.83); P = 0.17] were similar. IL-4, IL-10, and interferon-gammas were undetectable in both the groups. 18F-FDG-PET showed putaminal hypermetabolism in 7/9 (78%) and thalamo-cerebellar hypometabolism in 8/9 (88%) with variable foci of cortical hypometabolism.

Conclusions

Elevated CSF NFH levels suggest ongoing neuronal loss in SSPE. Cell mediated immunity is altered, and active neuroinflammation (T-cell mediated) may not be contributing to the ongoing neurodegeneration. 18F-FDG-PET imaging consistently reveals putaminal hypermetabolism alongside thalamo-cerebellar hypometabolism, which may prove valuable in diagnosing challenging SSPE cases.

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揭示亚急性硬化性全脑炎的炎症和代谢：一项前瞻性多模式评估。

背景：本研究讨论了亚急性硬化性全脑炎（SSPE）患者脑脊液（CSF）神经炎症和神经退行性变生物标志物的多模式评估结果和氟脱氧葡萄糖-正电子发射断层扫描（18F-FDG-PET）结果。方法：在研究期间（2020-2022年）连续招募符合Dyken标准的SSPE患儿（3-14岁）。记录临床资料、放射学和脑电图资料。CSF白介素（il） IL-4、IL-6、IL-10；移行细胞;和神经丝重链（NFH）水平进行分析，并与年龄匹配的伪对照进行比较。18F-FDG-PET成像在一组同意的儿童中进行。结果：33例SSPE患儿入组[平均年龄8.06±2.67岁；男性:82%;Jabbour阶段I-6%，阶段II-67%，阶段III-27%]。两种表现形式：短/无I期伴早发性肌阵挛痉挛（N = 22，平均发病年龄7.85±2.45）和延长I期伴晚发性癫痫（N = 11，平均年龄6±1.9岁，P = 0.03）。SSPE患者脑脊液NFH显著升高[132.62 (53.6-388.9)vs 0.0 (0-16.9) pg/ml；P = 0.0001]。CSF IL-6 （pg/ml）在21/22 SSPE患儿[1.72(0.85-3.04)]和16/17伪对照组[2.60 (1.72-3.83)]；P = 0.17]相似。两组均未检测到IL-4、IL-10和干扰素γ。18F-FDG-PET表现为7/9的壳层代谢高（78%），8/9的丘脑-小脑代谢低（88%），皮质代谢低的病灶不同。结论：脑脊液NFH水平升高提示SSPE患者神经元持续丢失。细胞介导的免疫被改变，活动性神经炎症（t细胞介导）可能不会导致持续的神经变性。18F-FDG-PET成像一致显示壳层代谢高和丘脑-小脑代谢低，这可能对诊断具有挑战性的SSPE病例有价值。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Pediatric neurology 医学-临床神经学

CiteScore

4.80

自引率

2.60%

发文量

176

审稿时长

78 days

期刊介绍： Pediatric Neurology publishes timely peer-reviewed clinical and research articles covering all aspects of the developing nervous system. Pediatric Neurology features up-to-the-minute publication of the latest advances in the diagnosis, management, and treatment of pediatric neurologic disorders. The journal''s editor, E. Steve Roach, in conjunction with the team of Associate Editors, heads an internationally recognized editorial board, ensuring the most authoritative and extensive coverage of the field. Among the topics covered are: epilepsy, mitochondrial diseases, congenital malformations, chromosomopathies, peripheral neuropathies, perinatal and childhood stroke, cerebral palsy, as well as other diseases affecting the developing nervous system.