Just a "Puff of Smoke"

Area of Science:

• Neuroscience
• Vascular Neurology
• Radiology

Background:

• Moyamoya disease is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries and the development of collateral vessels.
• It can lead to recurrent strokes, cognitive decline, and is associated with a hereditary component.
• Early diagnosis and intervention are critical for managing the condition and improving patient outcomes.

Purpose of the Study:

• To present a case of Moyamoya disease in a 44-year-old woman with a history of stroke and seizures.
• To highlight the diagnostic challenges and treatment implications in a patient unable to provide informed consent.
• To emphasize the importance of early diagnosis for timely intervention in Moyamoya disease.

Main Methods:

• Clinical presentation of a 44-year-old female with altered mental status and seizure-like activity.
• Neurological examination revealing lethargy, agitation, and nonsensical speech.
• Neuroimaging including computed tomography (CT) and CT angiogram demonstrating cerebral atrophy, encephalomalacia, edema, and occlusion of internal carotid arteries with a Moyamoya pattern.

Main Results:

• Initial CT showed left middle cerebral artery (MCA) territory infarct.
• Repeat CT revealed evolving infarcts in right posterior cerebral artery (PCA) and right MCA territories with edema.
• CT angiogram confirmed bilateral internal carotid artery stenosis, indicative of Moyamoya disease, with collateral flow.

Conclusions:

• The patient was diagnosed with Moyamoya disease, a condition with a poor prognosis and potential for cognitive decline.
• Cerebral bypass was recommended but could not be performed due to the patient's inability to consent and family refusal.
• The case underscores the critical need for early diagnosis of Moyamoya disease to enable timely treatment before patients become debilitated.