Moyamoya-like vasculopathy associated to MYH9-related thrombocytopenia manifested by multiple cerebral ischemic lesions: a case report.
BMC Neurol
; 20(1): 352, 2020 Sep 19.
Article
em En
| MEDLINE
| ID: mdl-32950057
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.Palavras-chave
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Base de dados:
MEDLINE
Assunto principal:
Trombocitopenia
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Ataque Isquêmico Transitório
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Cadeias Pesadas de Miosina
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Doença de Moyamoya
Idioma:
En
Ano de publicação:
2020
Tipo de documento:
Article