Department of Neuropediatrics, University Medical Center Schleswig-Holstein, Kiel University (CAU), Kiel, Germany.
Division of Neuropediatrics and Social Pediatrics, Department of Pediatrics, University Hospital, RWTH Aachen, Aachen, Germany.
Neuropediatrics. 2024 Feb;55(1):9-15. doi: 10.1055/s-0043-1774318. Epub 2023 Oct 5.
Describing spectrum, evolution, and clinical relationship of brain magnetic resonance imaging (MRI) findings in a large case series of children with febrile infection-related epilepsy syndrome (FIRES).
This retrospective study included 31 children with FIRES. Clinical data and MRI findings of the brain were evaluated. Poor clinical outcome was defined as severe disability, persistent vegetative state or stupor, very low intelligence quotient (<80), or death (modified Rankin scale 4-6 and Glasgow Outcome Score 1-3).
Seventeen (54.8%) children with FIRES showed no abnormalities in the initial MRI, whereas 28 (90.3%) children showed MRI abnormalities at follow-up. The most frequent abnormalities were brain atrophy (74.2%) and T2/fluid-attenuated inversion recovery changes (64.5%), mostly hippocampal (45.2%). Generalized brain atrophy was the most frequent type of atrophy (58%). The earliest atrophy was recorded 9 days after the onset of disease. It progressed even beyond the acute phase in most children (51.6%). The exploratory data analysis revealed nominal significance between all MRI abnormalities considered together and poor outcome ( = 0.049) and between generalized brain atrophy and anesthesia ( = 0.024). After adjustment for multiple testing, the values were not significant. The outcome in four (12.9%) children was not poor despite generalized brain atrophy.
In contrast to the uniform clinical course, MRI demonstrated a broad spectrum of findings. Initially, these were mostly normal and therefore indicative of FIRES but then changed rapidly and were mostly progressive despite the stable chronic course. The cause may be ongoing disease, treatment intensity, or both. Future studies should focus on what process underlies the onset and the progression of brain atrophy. However, brain atrophy was not always related to poor outcomes in children despite FIRES.
描述大量热性感染相关癫痫综合征（FIRES）患儿的脑磁共振成像（MRI）表现的范围、演变和临床相关性。
本回顾性研究纳入了 31 例 FIRES 患儿。评估了临床数据和脑部 MRI 表现。不良临床结局定义为严重残疾、持续性植物状态或昏迷、极低智商（<80）或死亡（改良 Rankin 量表 4-6 级和格拉斯哥结局量表 1-3 级）。
17 例（54.8%）FIRES 患儿初始 MRI 无异常，而 28 例（90.3%）患儿在随访时 MRI 异常。最常见的异常是脑萎缩（74.2%）和 T2/液体衰减反转恢复改变（64.5%），主要是海马（45.2%）。广泛性脑萎缩是最常见的萎缩类型（58%）。最早的萎缩发生在疾病发病后 9 天。在大多数儿童中，即使在急性期后，萎缩也会继续进展（51.6%）。探索性数据分析显示，考虑到所有 MRI 异常，与不良结局之间存在显著相关性（ = 0.049），与广泛性脑萎缩和麻醉之间存在显著相关性（ = 0.024）。在进行多重检验调整后，这些值不具有显著意义。尽管存在广泛性脑萎缩，仍有 4 例（12.9%）患儿的结局不不良。
与一致的临床病程相反，MRI 显示出广泛的表现范围。最初，这些异常主要是正常的，因此提示 FIRES，但随后迅速变化，尽管处于稳定的慢性病程中，但大多数呈进行性改变。原因可能是疾病的持续存在、治疗强度或两者兼而有之。未来的研究应集中于脑萎缩发生和进展的潜在过程。然而，尽管患有 FIRES，脑萎缩并不总是与儿童的不良结局相关。
