Sickle Cell Disease and Stroke
A 24-year-old man with sickle cell disease (SCD) was noncompliant with medication and presented in pain crisis with severe abdominal and back pain. He had no neurological complaints or abnormalities on neurological examination. Later, he was transferred to the Medical Intensive Care Unit with lethargy, reduced hemoglobin, and impaired kidney, liver, and heart function. The hemoglobin S percentage (HbS%) was 84, and he was transfused to reduce the HbS% to 55; however, his multiorgan failure worsened.
The next day he developed left arm weakness, but the time of onset was unclear. His examination showed a left hemiparesis and hemisensory loss. His National Institutes of Health stroke score was 10, and computed tomography of the head was unremarkable. Thrombolytics were not given because of the unknown onset. MRI of the brain showed scattered diffusion-positive lesions throughout both cerebral hemispheres. Magnetic resonance angiography of the head and neck showed no large vessel occlusion. Transesophageal echocardiogram was negative for a cardioembolic source of emboli, and telemetry records were unremarkable. He started aspirin 81 mg daily and underwent exchange transfusion. His HbS% stabilized at 23 to 25, and his neurological examination improved.
A few days later, he developed severe headache and had a generalized tonic clonic seizure. Computed tomography now showed a left frontal subarachnoid hemorrhage. He was treated with levetiracetam and underwent magnetic resonance angiography, magnetic resonance venography, and cerebral angiogram, which showed no aneurysms or other vascular malformations. His coagulation panel was unremarkable, but the HbS% was 16. Of note, imaging requiring iodinated dye was delayed until after his crisis resolved because of potential vasospastic complications from the contrast material during crisis.
The incidence of ischemic and hemorrhagic strokes is increased in adults with SCD relative to the general population. The prevalence of stroke is 3.75% in …