Moyamoya-like vasculopathy associated to MYH9-related thrombocytopenia manifested by multiple cerebral ischemic lesions:
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CASE REPORT
Open Access
Moyamoya-like vasculopathy associated to MYH9-related thrombocytopenia manifested by multiple cerebral ischemic lesions: a case report Athena Cristina Ribigan1,2,3* , Raluca Stefania Badea1,2,3, Alida Ciocan1, Dana Stefan1, Bogdan Casaru1, Patricia Ioan1, Florina Antochi1,3 and Ovidiu Băjenaru1,2,3
Abstract Background: Moyamoya-like vasculopathy (MMV) and myosin heavy chain 9-related platelet disorders (MYH9-RPDs) or macrothrombocitopenias are rare syndromes. Their association is even more infrequent. Case presentation: A 29-year-old female with history of MYH9-RPD, presented to our department for episodes suggesting transient ischemic attacks. Based on the imaging studies that revealed multiple ischemic lesions and stenoses of both distal internal carotid arteries and the arteries of the circle of Willis, the diagnosis of MMV was established. The treatment with Verapamil was initiated, leading to symptom remission. Two months later, the patient presented one episode of dysarthria, followed by involuntary movements of the right upper limb, few days later. Long-term electroencephalogram monitoring depicted epileptiform abnormalities. Resolution of symptoms was obtained after increasing the dose of Verapamil, and initiating Levetiracetam. Conclusions: This is an interesting case of a patient with two rare pathologies, who presented with cerebral ischemic strokes. To our knowledge there are few cases described in the literature presenting with cerebral hemorrhagic events but none of them with multiple cerebral ischemic lesions. As these cases are very rare, it is important to gather evidence regarding the best approach and treatment strategy. Keywords: Moyamoya, Vasculopathy, MYH9, Thrombocytopenia, Ischemic lesions
Background Moyamoya disease (MMD) is an idiopathic disorder defined by progressive occlusion or stenoses of the intracranial distal segments of the internal carotid arteries (ICAs) and the arteries of circle of Willis associated with the development of numerous collaterals suggesting an * Correspondence: [email protected] 1 Neurology Department, University Emergency Hospital Bucharest, Splaiul Independentei, number 169, district 5, 050098 Bucharest, Romania 2 Department of Clinical Neurosciences, University of Medicine and Pharmacy Carol Davila Bucharest, Dionisie Lupu street, number 37, district 1, 020021 Bucharest, Romania Full list of author information is available at the end of the article
aspect of “a hazy cloud like a puff of cigarette smoke” [1]. When the aforementioned abnormalities are present in patients with other concomitant diseases, the condition is called Moyamoya syndrome (MMS) or MMV due to the fact that the underlying disease may participate to the pathogenesis of the vasculopathy. Some of the conditions associated with MMS include hematologic disorders [2]. MYH9-RPD represent a group of hereditary macrothrombocytopenias characterized by thrombocytopenia, giant platelets, and a combination of leukocyte inclusion bodies, hearing loss, nephritis or cata
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