Reversible Diffusion-Restricting White Matter Lesions Associated with Pain Crisis in Sickle Cell Disease: A Case Report
Meyer Herzog1, Esewi Aifuwa1, Joseph Quintas2, Sana Ali3, Amir Steinberg2, Tomoko Kitago4
1New York Medical College, 2Hematology-Oncology, 3Radiology, 4Neurology, Westchester Medical Center
Objective:
N/A
Background:
Neurological and cerebrovascular complications are relatively common in patients with sickle cell disease (SCD). Three main categories include large vessel infarctions, silent infarctions, and hemorrhage. Here we present a rare case of reversible, diffuse white matter lesions associated with severe neurological dysfunction. 

Design/Methods:
N/A
Results:

An 18-year-old African American man with SCD presented with acute pain crisis and fevers. He was treated with antibiotics and analgesics but developed worsening hypoxemia and mental status, requiring intubation. MRI brain showed diffuse restricted diffusion and FLAIR hyperintensities symmetrically involving bilateral cerebral deep white matter and splenium of the corpus callosum. MRA head was unremarkable, EEG showed no seizures, and CSF analysis was unremarkable. The patient was treated with hydroxyurea, voxelotor, exchange transfusion, and analgesics. Following extubation, the neurological exam remained poor with no verbal output, inability to follow commands, and spastic quadriparesis. The patient was eventually discharged to a rehabilitation facility. Monthly exchange transfusions were continued. Three months later, his neuro exam showed marked clinical improvement and near-complete resolution of subcortical and splenial white matter abnormalities on MRI. Six months later he was back to his neurological baseline.


Conclusions:
SCD is known to cause several cerebrovascular complications. This case report highlights a rare complication of SCD that presents with severe neurologic dysfunction with cerebral vaso-occlusive crisis involving white matter that is reversible with treatment. 

10.1212/WNL.0000000000210951
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