An adolescent girl with Moyamoya disease: JAMA
A girl in her midteens presented to an ophthalmology practice with her parents reporting worsening blurry vision on the right side for the past 2 weeks. She had a history of migraine headaches, for which she took nonsteroidal anti-inflammatory drugs for symptomatic relief.

At her examination, she reported feeling tired but denied other medical problems. On examination, her visual acuity was 20/20 OU. Intraocular pressure was 15 mm Hg OU, extraocularmotility was normal, and findings of the anterior segment examination were normal. There was no afferent pupillary defect, and dilated fundus examination revealed healthy-appearing optic nerves and normal retinas.

Automated perimetry demonstrated a right-sided homonymous hemianopia. MRI of the brain performed the same day revealed subacute infarction of portions of the left occipital, temporal, and parietal lobes (Figure).

She was diagnosed with Moyamoya disease.

Moyamoya disease is a chronic, occlusive cerebrovascular disease of unknown etiology. Children with moyamoya disease typically present with transient ischemic attacks or strokes. Other causes of cerebral infarction, including atherosclerosis, autoimmune diseases, meningitis, and tumors, must be ruled out.

Key takeaways:-
• Definitive diagnosis of moyamoya disease can be made by magnetic resonance angiography or conventional catheter angiography

• The most characteristic finding in moyamoya disease is stenosis of the internal carotid arteries in conjunction with abundant collateral vessels as a result of the disease.

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