Mononeuropathy multiplex in sickle cell disease: a complication in need of recognition

Peripheral nervous system involvement is rare in sickle cell disease. A review of the literature has uncovered only a few reports of nerve deficit in association with sickle cell disease, and often a clear relationship between the neuropathy and the anemia is absent. Mononeuropathy resulting from peripheral nerve infarction, as a complication of sickle vaso-occlusive crisis, seemingly is uncommon and, to our knowledge, has been reported only once. We report two patients who developed acute mononeuropathy multiplex in the setting of sickle cell pain crisis. The clinical and electrodiagnostic findings were consistent with a multifocal nerve disorder resulting from an ischemic process caused by a sickle cell vaso-occlusive crisis. We describe the clinical course, electrophysiological findings, diagnosis, and management of these patients. We also analyze the anatomic and pathophysiological basis of this disorder and offer a possible clinical explanation for its infrequent and exceptional diagnosis.