Moyamoya disease is a chronic progressive cerebrovascular arteriopathy of unknown origin which is characterized by progressive stenosis internal carotid artery and cerebral arteries with typ angiographic appearance of abnormal basal collateral vessels. Clinical features include early neurological symptoms (eg. dysarthria, aphasia, hemiparesis and seizures) while visual impairment is considered atypical, being generally reported as a later complication of ischemic or hemorragic events. We report a case of a 4-years girl who developed an acute onset of right visual loss with normal eye movements, without any neurological and behavioural alterations. A magnetic resonance angiography showed features suggestive of Moyamoya disease with a typical “puff of smoke” aspect con?irmed with an angiography: it was started heparin and antiepileptic medications and after she was subject to two surgical intervention (indirect EDAS revascularization combined with right EDAMPS) with instrumental and clinical improvement. Moyamoya disease should be included in differential diagnosis of each pediatric patient with acute onse visual loss: delay of diag
Acute Onset Visual Loss: Presentation of Moyamoya Disease in a Girl
DI CARA, GIUSEPPE;PANFILI, ELISA;Verrotti, Alberto;
2016
Abstract
Moyamoya disease is a chronic progressive cerebrovascular arteriopathy of unknown origin which is characterized by progressive stenosis internal carotid artery and cerebral arteries with typ angiographic appearance of abnormal basal collateral vessels. Clinical features include early neurological symptoms (eg. dysarthria, aphasia, hemiparesis and seizures) while visual impairment is considered atypical, being generally reported as a later complication of ischemic or hemorragic events. We report a case of a 4-years girl who developed an acute onset of right visual loss with normal eye movements, without any neurological and behavioural alterations. A magnetic resonance angiography showed features suggestive of Moyamoya disease with a typical “puff of smoke” aspect con?irmed with an angiography: it was started heparin and antiepileptic medications and after she was subject to two surgical intervention (indirect EDAS revascularization combined with right EDAMPS) with instrumental and clinical improvement. Moyamoya disease should be included in differential diagnosis of each pediatric patient with acute onse visual loss: delay of diagI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
