Moyamoya Syndrome with Recurrent Stroke in a Splenectomised Patient with BetaThalassaemia Major: A Case Report

Moyamoya Syndrome (MMS) is a progressive disease with typical angiographic ‘moyamoya’ alterations. Here, the authors discuss a case of 16-year-old male patient who developed MMS after being diagnosed with β-thalassaemia major. This patient was detected with heterozygous mutations in the β-globin gene and underwent splenectomy at the age of 12 years. Four years postsplenectomy he presented with paresis of the right upper limb. Magnetic Resonance Imaging (MRI) showed infarction and stenosis of the internal carotid artery with the collateral vessel’s formation. Recurrence of stroke and progression of the vasculopathy were seen. Till now, only a few cases of MMS have been linked to thalassaemia major.