A Rare Case of Moyamoya Disease in a Patient With Sickle Cell Disease

Area of Science:

• Neurology
• Vascular Neurology
• Radiology

Background:

• Moyamoya disease (MMD) is a rare, progressive cerebrovascular disorder characterized by stenosis of the internal carotid arteries and the development of abnormal collateral vessels.
• It predominantly affects individuals of East Asian descent but can occur in other populations.
• MMD can lead to severe neurological deficits such as stroke and seizures.

Observation:

• This report focuses on a 32-year-old African-American female with a history of sickle cell disease and stroke.
• She presented with worsening left-sided weakness and left wrist drop.
• Despite an early MMD diagnosis, prior medical records were unavailable.

Findings:

• Brain imaging confirmed Moyamoya disease, revealing characteristic minute collateral vessels replacing the middle cerebral artery.
• The patient's presentation of left-sided hemiparesis and wrist drop was consistent with MMD-related ischemic events.
• The case underscores the importance of considering MMD in patients with relevant risk factors and neurological symptoms, even with limited historical data.

Implications:

• This case highlights the importance of recognizing Moyamoya disease in diverse populations, including African Americans.
• It emphasizes the need for comprehensive diagnostic evaluation, including advanced brain imaging, for patients presenting with unexplained neurological deficits.
• Early diagnosis and management of MMD are crucial for preventing progressive neurological damage and improving patient outcomes.