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Acute Neurological Events in Children With Hemoglobin SC Disease: A Multicenter Retrospective Study
Célia Paulmin1, Bérengère Koehl2, Audrey Maudoux3
1Le Kremlin Bicêtre, Reference Center for Rare Constitutional Disorders of the Red Blood Cell and Erythropoiesis (MCGRE), Bicêtre Hospital, Le Kremlin-Bicêtre, France.
Insights
Acute neurological events are common in children with hemoglobin SC (HbSC) disease, with neuro-ENT issues being most frequent. These events present a diverse range of symptoms, highlighting the need for further research in pediatric HbSC disease management.
Area of Science:
- Pediatric Neurology
- Hematology
- Sickle Cell Disease Research
Background:
- Neurological complications in pediatric hemoglobin SC (HbSC) disease are not well-understood, especially acute events.
- Characterizing the range and incidence of these events is crucial for patient care.
Purpose of the Study:
- To delineate the spectrum and frequency of acute neurological events in children with HbSC disease.
- To analyze data from a multicenter cohort to provide a comprehensive overview.
Main Methods:
- Retrospective descriptive multicenter study.
- Inclusion of children aged 0-18 years with HbSC disease experiencing at least one acute neurological event.
- Data collection period from January 1, 2010, to August 31, 2023, across specified French regions.
Main Results:
- Twenty-five pediatric patients from 10 centers experienced 34 acute neurological events.
- Neuro-ENT manifestations (vestibular syndromes, hearing loss) were most common (41%), followed by central neurological deficits/altered consciousness (18%), seizures (18%), headaches (18%), and peripheral neuropathies (6%).
- Cerebrovascular events occurred across all ages, particularly in those with a history of vaso-occlusive crises; some neuro-ENT events recurred.
Conclusions:
- Acute neurological events in children with HbSC disease are heterogeneous.
- Further research is essential to understand the triggers and clinical impact of these neurological events in pediatric HbSC disease.
Introduction:
Neurological manifestations in children with hemoglobin SC (HbSC) disease remain insufficiently characterized, particularly regarding acute events. The aim of this study was to describe the spectrum and frequency of acute neurological events in a multicenter cohort of children with HbSC disease.
Materials And Methods:
We conducted a retrospective descriptive multicenter study including children with HbSC disease aged 0-18 years who presented with at least one acute neurological event between January 1, 2010, and August 31, 2023, in Île-de-France, Martinique, and Guadeloupe.
Results:
Twenty-five patients from 10 centers were included. A total of 34 acute neurological events were recorded. Neuro-ENT (ear, nose, throat) manifestations were the most frequent (41%), including vestibular syndromes and sudden hearing loss, followed by central neurological deficits or altered consciousness (18%), seizures (18%), acute headaches (18%), and peripheral neuropathies (6%). Neuro-ENT manifestations showed recurrence in some patients. Cerebrovascular events occurred across all age groups and were observed in patients with a history of vaso-occlusive crises.
Conclusion:
Acute neurological events may occur in children with HbSC disease and show a heterogeneous clinical spectrum. Further studies are needed to better characterize their determinants and clinical significance.
Clinical Trial Registration:
Not applicable.
