[Moyamoya disease with persistent primitive hypoglossal artery. Case report]

Area of Science:

• Neurology
• Vascular Surgery
• Neuroimaging

Background:

• Moyamoya disease is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries and the development of abnormal collateral vessels.
• Persistent primitive hypoglossal artery is a rare congenital anomaly representing a remnant of the embryonic hypoglossal artery.

Observation:

• A 44-year-old female presented with aphasia and left eye visual disturbance.
• Four-vessel angiography revealed right internal carotid artery occlusion, left carotid fork stenosis, bilateral moyamoya vessels, and a left-sided persistent primitive hypoglossal artery.
• Cerebral blood flow measurements indicated reduced flow in the left hemisphere, unresponsive to acetazolamide loading.

Findings:

• The patient underwent successful superficial temporal artery-middle cerebral artery anastomosis on both sides.
• This represents the first reported case of moyamoya disease co-occurring with a persistent primitive hypoglossal artery.
• The findings suggest that while not etiologically linked, a persistent primitive hypoglossal artery may offer a collateral pathway in moyamoya disease.

Implications:

• The coexistence of moyamoya disease and persistent primitive hypoglossal artery expands the understanding of cerebrovascular anomalies.
• The persistent primitive hypoglossal artery may play a role in cerebral collateral circulation, potentially influencing surgical considerations.
• Further research is needed to elucidate the relationship and clinical significance of this rare association.