[P1-215] Spectrum of Clinical Presentation In Pediatric Moyamoya Disease : A Case Series
Moyamoya disease MMD, an occlusive cerebrovascular disorder characterised by stenosis of the distal internal carotid arteries ICAs and the development of compensatory collateral vessels, is increasingly recognised as an entity associated with pediatric stroke. Although ischemic episodes in the form of transient ischemic attacks TIAs or strokes are the predominant events leading to moyamoya diagnosis, children with moyamoya may present with other signs and symptoms suggestive of the diagnosis.
We reviewed the clinical presentation in a case series of pediatric MMD . From a cohort study of pediatric stroke over a 15-year period, we identified 9 children with MMD, 5 males and 4 females , mean age 9.2 years (range 5-14). One child had Down Syndrome; a second child had Neurofibromatosis type 1. Presenting symptoms were: TIAs (4), acute ischemic stroke (3), headache (2), seizures (2), acute febrile illness with focal neurological deficits (2), language impairment (1) and movement disorder (1). Cranial magnetic resonance imaging with angiography and four-vessel cerebral angiograms confirmed moyamoya disease. Bilateral ICA involvement was seen in all cases except in the child with Neurofibromatosis type 1. Management consisted of antiplatelet therapy and encephalo-duro-arterio-synangiosis. Post-surgical follow-up of 8 children revealed no stroke recurrence.
Our case series reflects the spectrum of clinical presentation in children with moyamoya disease. Prompt diagnosis and appropriate intervention are crucial in improving prognosis of this progressive cerebrovasculopathy. MMD should be considered, and diagnostic evaluation initiated, in children presenting with neurologic deficits or unexplained symptoms suggestive of cerebral ischemia.
We reviewed the clinical presentation in a case series of pediatric MMD . From a cohort study of pediatric stroke over a 15-year period, we identified 9 children with MMD, 5 males and 4 females , mean age 9.2 years (range 5-14). One child had Down Syndrome; a second child had Neurofibromatosis type 1. Presenting symptoms were: TIAs (4), acute ischemic stroke (3), headache (2), seizures (2), acute febrile illness with focal neurological deficits (2), language impairment (1) and movement disorder (1). Cranial magnetic resonance imaging with angiography and four-vessel cerebral angiograms confirmed moyamoya disease. Bilateral ICA involvement was seen in all cases except in the child with Neurofibromatosis type 1. Management consisted of antiplatelet therapy and encephalo-duro-arterio-synangiosis. Post-surgical follow-up of 8 children revealed no stroke recurrence.
Our case series reflects the spectrum of clinical presentation in children with moyamoya disease. Prompt diagnosis and appropriate intervention are crucial in improving prognosis of this progressive cerebrovasculopathy. MMD should be considered, and diagnostic evaluation initiated, in children presenting with neurologic deficits or unexplained symptoms suggestive of cerebral ischemia.