Related Experiment Video
Updated: Aug 8, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Moyamoya Syndrome (MMS) in a Patient With Sickle Cell Disease (SCD) and Protein S Deficiency
Parima Saxena1, Hussam Alkaissi2,3,1, Riddhi Chauhan4
1Internal Medicine, State University of New York Downstate Medical Center, Brooklyn, USA.
Insights
Moyamoya syndrome (MMS) in adults with sickle cell disease (SCD) is rare. This case study shows medical management, not surgery, can benefit adult SCD patients with MMS and protein S deficiency.
Area of Science:
- Neurology
- Hematology
- Vascular Medicine
Background:
- Moyamoya syndrome (MMS) association with sickle cell disease (SCD) is documented in children.
- Limited data exists on MMS characteristics and management in adult SCD patients.
- Current guidelines for stroke prevention in pediatric MMS are lacking for adults.
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.
More Related Videos
08:23Characterization of Sickling During Controlled Automated Deoxygenation with Oxygen Gradient Ektacytometry
Published on: November 5, 2019
05:23Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Related Concept Videos
Multiple Allele Traits
Overview of Protein Metabolism
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
Disorders of Erythrocytes
Erythrocyte disorders can be broadly categorized into two main types: anemic and polycythemic conditions.
A low oxygen-carrying capacity of the blood due to the loss, lower production, or destruction of erythrocytes is termed anemia. Hemorrhagic anemia, for example, occurs when bleeding from an external wound or internal ulcer reduces erythrocyte counts.
On the other...
Disorders of the Skeletal Muscle
Musculoskeletal disorders
Musculoskeletal disorders involve injuries and conditions affecting the skeletal muscles and associated connective tissues. These disorders can arise from acute biomechanical stresses or chronic overuse and can occur across different age groups. Common injuries include sprains, fractures, and muscular strains, often resulting from...
Inborn Errors of Metabolism
Peripheral Arterial Disease II: Clinical Manifestations and Diagnostic Evaluation