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Case Reports in Hematology
Volume 2015, Article ID 470873, 3 pages
Case Report

HbSC Disease and Spontaneous Epidural Hematoma with Kernohan’s Notch Phenomena

1Department of Medicine, Interfaith Medical Center, Brooklyn, NY 11213, USA
2Department of Neurosurgery, North Shore-LIJ Health System, Manhasset, NY 11030, USA

Received 1 September 2015; Accepted 4 October 2015

Academic Editor: Gergely Feher

Copyright © 2015 Meera Yogarajah et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Spontaneous (nontraumatic) acute epidural hematoma is a rare and poorly understood complication of sickle cell disease. A 19-year-old African American male with hemoglobin SC disease (HbSC) presented with generalized body aches and was managed for acute painful crisis. During his hospital stay he developed rapid deterioration of his mental status and computed topography revealed a spontaneous massive epidural hematoma with mass effect and midline shift with Kernohan’s notch phenomena for which urgent craniotomy and evacuation was done. We report the first case of HbSC disease associated with catastrophic epidural hematoma progressing to transtentorial herniation and Kernohan’s notch phenomena within few hours with rapid clinical deterioration. The etiopathogenesis and the rare presentation are discussed in detail in this case report.