Myelopathy in sickle cell disease: a case-oriented review

Area of Science:

• Neurology
• Hematology
• Spinal Cord Medicine

Background:

• Neurological complications are common in sickle cell disease (SCD).
• Myelopathy, particularly compressive and ischemic types, is rarely described in SCD.
• This study introduces longitudinally extensive myelitis (LETM) as a potential neurological complication in SCD.

Observation:

• A 29-year-old man with SCD presented with subacute flaccid paraparesis, urinary retention, and a sensory level.
• Cerebrospinal fluid analysis revealed lymphocytic pleocytosis and elevated protein.
• Spinal MRI showed a T2/STIR hyperintense lesion extending from C2 to T12.

Findings:

• Reviewed literature identified few cases of spinal cord compression and infarction in SCD.
• Only one case of varicella-zoster myelitis was reported.
• This is the first reported case of LETM in a patient with SCD.

Implications:

• LETM should be considered in the differential diagnosis of myelopathy in SCD patients.
• Prompt diagnosis and management of myelopathy are crucial in SCD.
• Recognizing LETM expands the spectrum of spinal cord complications in SCD.