Objective:

To describe neurological complications in sickle cell and sickle cell trait across a 17-patient series: emphasizing seizure burden, cerebrovascular events, cerebral edema, and the rationale for the need of biomarker-guided neuroimaging in the African American demographic.

Background:

In our cohort, neurologic sickle cell complications caused substantial disability among African Americans. Pediatric stroke screening is routine, yet adult cases often presented with refractory seizures, hemorrhage, and edema: patterns underappreciated in literature. Emerging studies link neurofilament light chain (NfL), brain-derived neurotrophic factor (BDNF), and MRI metrics (oxygen extraction fraction, diffusion indices) to subclinical injury, but further research is required.

Design/Methods:

We retrospectively reviewed 17 consecutive sickle cell patients (15 SCD, 2 SCT; mean age 45.5 years; 9 males, 8 females) with neurological complications. Demographics, genotype, presentation, neuroimaging, treatments, and outcomes were abstracted: grouped by complication type and  recurrent patterns.

Results:

Ten of 17 patients (58.8%) had seizures, often recurrent, and treatment-resistant. Breakthrough seizures were closely linked to recent transfusions and nonadherence. Ischemic stroke occurred in 4 patients, hemorrhagic stroke in 2, and silent cerebral infarcts in 9/17 (53%) on MRI. Cerebral edema developed in 2 patients with poor outcomes. Additional diagnoses included moyamoya (2), posterior reversible encephalopathy (1), autoimmune encephalitis/lupus (1), and cerebral venous sinus thrombosis (1). Pediatric cases inclined towards ischemia while adults had higher hemorrhage rates and cognitive decline. Research shows biomarker analysis of elevated NfL with higher infarct burden and recurrent seizures; BDNF alterations correlated with recurrent ischemia. Advanced MRI showed impaired oxygen extraction and white matter injury before massive infarcts formed. Chronic transfusion reduced recurrence risk; hydroxyurea showed partial benefit.

Conclusions:

Seizures were the dominant neurologic complication, with cerebral edema as a catastrophic, underrecognized event. Imaging correlations suggest potential for earlier risk detection and intervention with further biomarker research needed. Multidisciplinary care, adherence to disease-modifying therapy, and aggressive seizure management remain essential.