(Kiosk 4) When Sickle Cell Takes a Paralyzing Turn: A Rare Spinal Cord Injury

Case Diagnosis: Intrathecal hemorrhage leads to T10 complete paraplegia secondary to sickle cell disease

Case Description or Program Description: Mr. Blue is a 23-year-old male with a past medical history of sickle cell disease (hbSC) with two recent ED visits for sickle cell crises, and alcohol use disorder who presented to the emergency room in February 2022 with lower extremity pain, weakness, and urinary retention after a session of heavy lifting. He described the pain as a burning sensation down both legs. On physical exam, he had full strength in bilateral upper extremities and no strength in bilateral lower extremities, with absent quadriceps and Achilles reflexes.

Setting: Inpatient Rehab

Assessment/Results: Thoracic and lumbar MRI showed T11-L1 cord compression secondary to intrathecal hematoma with hypodense edema in the lumbar and upper sacral spine. Spinal and brain angiograms showed no arterial or venous malformations. Neurosurgery deferred surgery. He started IV Decadron for swelling, and gabapentin for radicular pain. Upon rehabilitation assessment, he had a T10 ASIA complete spinal cord injury (SCI) and was admitted to acute rehabilitation for bowel and bladder training, along with physical and occupational therapy.

Discussion (relevance): Sickle cell patients have a risk of spontaneous cerebral vascular incidents, with the highest age-adjusted prevalence estimate being 4.01% in SS allele variant, 2.43% in S-β0 thalassemia, 1.29% in S-β+thalassemia, and 0.84% in SC allele variant. Most commonly intrathecal hemorrhages occur due to trauma, vascular malformation, or iatrogenic causes. However, Mr.Blue had a stable INR, no arteriovenous malformations, nor any recent sheering traumas. Mr.Blue's highest risk for an incident was his SC allele variant sickle cell disease.

Conclusions: Although there are cases of spontaneous hemorrhages causing spinal cord injury, this is a rare case where a sickle cell event was the most likely etiology of the intrathecal hemorrhage that led to subsequent cord compression, cauda equina syndrome, and finally complete T10 paraplegia.