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J Pediatr Neurosci. 2018 Oct-Dec;13(4):514-516. doi: 10.4103/JPN.JPN_85_18.

Moyamoya Disease Presenting as Alternating Hemiparesis with Relapsing Remitting Hemichorea: An Unusual Manifestation.

Author information

1
Department of Neurology, Institute of Human Behaviour and Allied Sciences, New Delhi, India.
2
Division of cardiology, Atlanta VA Medical Center, Decatur, Georgia, USA.

Abstract

Case:

Moyamoya disease (MMD) is a neurological disease involving internal carotid artery (ICA) leading to its occlusion. Among the children, the disease presents as ischemic strokes, whereas in adults, it presents as hemorrhagic strokes. Movement disorder among the MMD is very rare with varied presentation. This article reports a case of 16-year-old girl presented with a history of alternating hemiparesis with recurrent hemichorea with self-remitting tendency. Magnetic resonance angiography brain showed marked-to-complete attenuation of supraclinoid ICA with multiple tortuous collateral vessels replacing the circle of Willis.

Conclusion:

MMD should be kept in the differential diagnosis of children presenting with alternating focal neurological deficit with recurrent movement disorder as movement disorder could be the initial presentation. MMD may present as waxing and waning features of the chorea and neurological deficit.

KEYWORDS:

Cerebral arterial diseases; Moyamoya disease; infarct; intracranial arterial diseases; movement disorder

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