Moyamoya disease in a Moroccan baby: a case report

Abstract

BackgroundA stroke in a baby is uncommon, recent studies suggested that their incidence is rising. Moyamoya disease is one of the leading causes of stroke in babies. This condition is mostly described in Japan. In Morocco, moyamoya disease has rarely been reported and a few cases were published. We report a rare Moroccan case of a 23-month-old baby boy who presented with left-sided hemiparesis and was diagnosed as having moyamoya disease.Case presentationA 23-month-old full-term Moroccan baby boy born to a non-consanguineous couple was referred to our hospital with the complaint of sudden onset left-sided hemiparesis. On neurological examination, there were no signs of meningeal irritation, his gait was hemiplegic, tone was decreased over left side, power was 2/5 over left upper and lower limb, and deep tendon reflexes were exaggerated. Preliminary neuroimaging suggested an arterial ischemic process. Clinical and laboratory evaluation excluded hematologic, metabolic, and vasculitic causes. Cerebral angiography confirmed the diagnosis of moyamoya disease. Our patient was treated with acetylsalicylic acid 5 mg/kg per day and referred to follow-up with pediatric neurosurgeon. Cerebral revascularization surgery using encephaloduroarteriosynangiosis was performed. At 8-month follow-up, his hemiparesis had improved and no further ischemic events had occurred.ConclusionThis case highlights the importance of considering moyamoya disease to be one of the classic etiologies of acute ischemic strokes in children from North Africa. It also emphasizes the rare presentation among the African population and the use of neurovascular imaging techniques to facilitate diagnosis of moyamoya disease.

Moyamoya Disease—“A Puff of Smoke”: A Rare Pediatric Case Report

“…The clinical features of the present case included dysarthria, aphasia, stroke, and hemiparesis on the right side. This is in accordance to the cases reported by Goyal et al, 16 Kim, 13 Okhovat and Moeini, 17 Janda et al, 18 and Houba et al 19 …”

Fascinating Case Presentation of Moyamoya Disease in Children and Adults

“…It has been seen that seizures and epileptiform electroencephalogram (EEG) changes are common in patients with MMD, as seen in our cases. In children, the re-build-up of slow waves at the end of hyperventilation has been said to be characteristic of MMD [ 2 , 6 ]. Hyperventilation-induced reduction in cerebral blood flow may be responsible for the recurrent TIAs seen in pediatric cases, for example when a child is excited, crying, or forcefully blowing on candles.…”

“…The MRA will show classic narrowing of the distal internal carotid artery and/or arteries around the circle of Willis with collaterals. Multiple tiny dot-like flow voids can be noted in the basal ganglia region (due to lenticulostriate and thalamoperforator collaterals) on T2WI and T1WI that enhance on contrast administration [ 6 ]. Bright sulci noted on T2 fluid-attenuated inversion recovery (FLAIR) and post-contrast T1WI form the ‘ivy sign’ due to the engorged pial collateral vessels (with slow blood flow) and thickened arachnoid membrane.…”

“…An MRI vessel wall imaging can distinguish MMD from moyamoya syndrome. Moyamoya disease typically shows stenosed distal ICA with no significant contrast enhancement without outer wall remodeling as compared to atherosclerosis (outer wall thickening in the stenosed segment compared to the proximal normal segment) or vasculitis (concentric wall thickening showing homogeneous enhancement) [ 6 ].…”

Surgery for Moyamoya Disease in Children

“…The use of multiple burr holes through frontal, parietal, and occipital bones has also been shown to provide adequate collateralization with clinical improvement and has been used as primary isolated revascularization, as an add-on to other techniques and as a rescue procedure. [88][89][90][91] This is also one of the least technically demanding revascularization techniques and can be done even under local anesthesia, potentially an attractive option in highsurgical-risk patients. 88 A meta-analysis of 57 publications detailing 1156 pediatric moyamoya disease patients who underwent a total of 1448 revascularization procedures (25% direct, 75% indirect) reported that 87% of those undergoing some form of surgical revascularization had symptomatic improvement.…”