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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.
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.
