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. 2023 Jul;30(7):2051-2061.
doi: 10.1111/ene.15821. Epub 2023 May 10.

Clinical and radiological description of 120 pediatric stroke-like episodes

Chloe Durrleman  1 , David Grevent  2   3 , Melodie Aubart  1 , Manoelle Kossorotoff  1 , Charles-Joris Roux  2 , Anna Kaminska  4 , Marlene Rio  5 , Giulia Barcia  5 , Nathalie Boddaert  2   3 , Arnold Munnich  5 , Rima Nabbout  1 , Isabelle Desguerre  1
Affiliations

Clinical and radiological description of 120 pediatric stroke-like episodes

Chloe Durrleman et al. Eur J Neurol. 2023 Jul.

Abstract

Background and purpose: Stroke-like episodes (SLEs) are defined as acute onset of neurological symptoms mimicking a stroke and radiological lesions non-congruent to vascular territory. We aimed to analyze the acute clinical and radiological features of SLEs to determine their pathophysiology.

Methods: We performed a monocenter retrospective analysis of 120 SLEs in 60 children over a 20-year period. Inclusion criteria were compatible clinical symptoms and stroke-like lesions on brain magnetic resonance imaging (MRI; performed for all 120 events) with focal hyperintensity on diffusion-weighted imaging in a non-vascular territory.

Results: Three groups were identified: children with mitochondrial diseases (n = 22) involving mitochondrial DNA mutations (55%) or nuclear DNA mutations (45%); those with other metabolic diseases or epilepsy disorders (n = 22); and those in whom no etiology was found despite extensive investigations (n = 16). Age at first SLE was younger in the group with metabolic or epilepsy disorders (18 months vs. 128 months; p < 0.0001) and an infectious trigger was more frequent (69% vs. 20%; p = 0.0001). Seizures occurred in 75% of episodes, revealing 50% episodes of SLEs and mainly leading to status epilepticus (90%). Of the 120 MRI scans confirming the diagnosis, 28 were performed within a short and strict 48-h period and were further analyzed to better understand the underlying mechanisms. The scans showed primary cortical hyperintensity (n = 28/28) with decreased apparent diffusion coefficient in 52% of cases. Systematic hyperperfusion was found on spin labeling sequences when available (n = 18/18).

Conclusion: Clinical and radiological results support the existence of a vicious circle based on two main mechanisms: energy deficit and neuronal hyperexcitability at the origin of SLE.

Keywords: children; energy deficit; mitochondrial disorder; neuronal hyperexcitability; stroke-like episodes.

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References

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