18º Congresso Brasileiro de Neurologia Infantil

Dados do Trabalho



Apresentação do caso

This case is about a previously healthy 7-year-old girl who, at the age of 3 years and 5 months, presented with aphasia and motor deficit in her right upper limb, followed by gelastic seizures. Imaging tests, including a Computadorized tomography, Magnetic Resonance Angiography and Angioresonance of the skull, revealed tapering of the supraclinoid portion of the internal carotid arteries, middle cerebral arteries, and initial segments of the anterior cerebral arteries, indicating Moyamoya disease and ischemic stroke. Two months later, the patient returned with sudden recurrent neurological deficits, dysphagia, sialorrhea, mental confusion, psychomotor agitation, and Broca's aphasia and was treated with diazepam and valproate. After two years, she presented with flaccidity, grade IV strength, altered tactile and painful sensibility in the left upper limb and central facial paresis on the right. She experienced a focal convulsive crisis, sialorrhea and left hemiparesis, which recurred during hospitalization and were treated with diazepam. She was diagnosed with a new ischemic stroke and aspirin was initiated.


Moyamoya is a Japanese term meaning "puff of smoke" - a neuroimaging description of the appearance of abnormal lenticulostriate collateral vessels that results from the progressive stenosis of the intracranial internal carotid circulation that characterizes the disease. Although it has no defined etiology, it has been associated with genetic conditions, exposure to radiation, vasculitis and infection. Moyamoya syndrome is the term used when it develops secondary to an underlying disease. The main clinical manifestations include transient ischemic attack or ischemic stroke, seizures, hemiparesis, altered level of consciousness, speech and sensory disturbances. Other symptoms may include hemorrhage of the vascular neoformation and headache. According to the Suzuki staging system, the circulatory pattern seen in the patient's angioresonance would be classified as stage 3, in which there is an increase in Moyamoya vessels and stenosis of the internal carotid artery. There is no specific treatment, and management is symptomatic. Interventional treatments include surgery, direct and indirect bypass, and encephaloduroarteriosynangiosis.

Comentários finais

A greater understanding of the pathophysiology and scientific advances in specific treatment are essential to achieve further medical outcomes. Specialized management is necessary for patients with Moyamoya disease.

Referências (se houver)

Palavras Chave

Moyamoya disease; Ischemic stroke; Gelastic seizures; Cerebrovascular disorders; Intracranial arterial diseases

Fonte de Fomento (se houver)

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Doenças cerebrovasculares e terapia intensiva em neurologia infantil


Universidade do Vale do Sapucaí - Minas Gerais - Brasil


Jessica Ariane Silva Valverde, Luisa Ribeiro Dias Godoy, Heder Atsushi Yaguchi, Caio Augusto de Martha Santos, Gustavo Belani Piolli, Julia Ribeiro Dias Godoy