*Correspondence: luisardgodoy@gmail.com.
Abstract
Case Presentation: 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.
Discussion: 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.
Final Comments: A greater understanding of pathophysiology and scientific advances in specific treatment
are essential to achieve further medical outcomes. Specialized management is necessary
for patients with Moyamoya disease.
