Unilateral Basal Ganglia Infarcts: a Red Flag for Ipsilateral Cranio-Cervical Arterial Occlusive Disease. A Report on...

Area of Science:

• Neurology
• Vascular Neurology
• Pediatric Neurology

Background:

• Moya-Moya disease is characterized by steno-occlusive disease in the internal carotid arteries and/or circle of Willis.
• It typically presents with a symmetric, bilateral pattern, leading to the formation of collateral perforator vessels visible as a 'puff of smoke' on angiography.
• The condition can lead to ischemic strokes due to under-perfusion of brain parenchyma.

Purpose of the Study:

• To describe two pediatric cases of Moya-Moya disease with exclusively unilateral macroangiopathy.
• To identify potential red flags for unilateral Moya-Moya disease in children.

Main Methods:

• Case report of two 11-year-old girls diagnosed with Moya-Moya disease.
• Detailed clinical presentation, neuroimaging findings (cerebral angiogram), and associated conditions (Down's syndrome, idiopathic).
• Comparison of the unilateral arteriopathy with the classic bilateral presentation of Moya-Moya disease.

Main Results:

• Both patients presented with unilateral internal carotid artery and ipsilateral middle cerebral artery macroangiopathy.
• One patient had Down's syndrome, the other had an idiopathic presentation.
• The unilateral arteriopathy differed from the typical symmetric and bilateral pattern of Moya-Moya disease.
• Both cases showed predominant deep gray matter (basal ganglia) strokes with ipsilateral parainsular infarcts.

Conclusions:

• Unilateral macroangiopathy of the internal carotid artery and middle cerebral artery can occur in Moya-Moya disease, differing from the classic bilateral presentation.
• The association of deep gray matter strokes with ipsilateral parainsular infarcts may serve as a potential red flag for unilateral Moya-Moya disease in children.