Moyamoya Syndrome (MMS) in a Patient With Sickle Cell Disease (SCD) and Protein S Deficiency

Parima Saxena; Hussam Alkaissi; Riddhi Chauhan; John Muthu

doi:10.7759/cureus.34314

Moyamoya Syndrome (MMS) in a Patient With Sickle Cell Disease (SCD) and Protein S Deficiency

Cureus. 2023 Jan 28;15(1):e34314. doi: 10.7759/cureus.34314. eCollection 2023 Jan.

Authors

Parima Saxena¹, Hussam Alkaissi^{2

3

1}, Riddhi Chauhan⁴, John Muthu⁵

Affiliations

¹ Internal Medicine, State University of New York Downstate Medical Center, Brooklyn, USA.
² Internal Medicine, Kings County Hospital Center, Brooklyn, USA.
³ Internal Medicine, Veterans Affairs Medical Center, Brooklyn, USA.
⁴ Internal Medicine, State University of New York Downstate Health Sciences University, Brooklyn, USA.
⁵ Kings County Hospital Center, Medicine, Sickle Cell Division, Brooklyn, USA.

Abstract

The association between Moyamoya syndrome (MMS) and sickle cell disease (SCD) has been well-established in pediatric populations; however, limited literature exists documenting the characteristics and management of MMS in adult SCD patients. Studies have indicated the role of endovascular management in secondary stroke prevention for pediatric populations, with no current guidelines available for adult populations. Here, we describe a unique case of MMS in a 30-year-old patient with SCD and incidental protein S deficiency. Our unique case highlights a patient at high risk for neurosurgical intervention due to her hypercoagulable state who has benefitted from medical management. We also discuss current literature for the prevention of secondary cerebral vascular events and the role of further studies involving adult populations with MMS and SCD.

Keywords: endovascular surgery; hereditary protein s deficiency; moyamoya disease (mmd); secondary stroke prevention; sickle cell disease: scd.

Publication types

Case Reports