Moyamoya Disease symptoms in children
Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing or occlusion of the arteries at the base of the brain, particularly the internal carotid arteries and their main branches. This narrowing causes the development of tiny, fragile blood vessels that attempt to compensate for reduced blood flow, creating a network that resembles a “puff of smoke” on imaging studies — hence the name “moyamoya,” which means “hazy” or “puff of smoke” in Japanese. While Moyamoya disease can affect individuals of all ages, it is notably significant in children because of its distinctive symptoms and the impact it can have on their development.
In children, Moyamoya often presents with symptoms related to stroke or transient ischemic attacks (TIAs), which are brief episodes of neurologic dysfunction caused by temporary blood flow reduction to parts of the brain. One of the earliest signs is sudden weakness or numbness on one side of the body, often affecting the face, arm, or leg. This weakness may occur intermittently, lasting for minutes to hours, and is often mistaken for other less serious conditions. Children may also experience speech difficulties, such as slurred speech or difficulty finding words, which can be alarming for parents and caregivers.
Another prominent symptom in pediatric cases is recurrent TIAs, which might be subtle or easily overlooked. These episodes may involve dizziness, visual disturbances, or brief lapses in consciousness. Because children might not always be able to articulate their symptoms clearly, caretakers should be vigilant for behavioral changes or signs of neurological distress, such as irritability, lethargy, or unsteady gait.
In some cases, Moyamoya disease manifests through more chronic symptoms related to cognitive and developmental delays. Due to reduced blood flow over time, children may experience difficulties with learning, concentration, or coordination. These subtle signs can sometimes be misdiagnosed as behavioral issues or other developmental disorders, delaying proper diagnosis and treatment.
In addition to ischemic symptoms, hemorrhagic events, such as sudden bleeding in the brain, may occur due to rupture of the fragile abnormal vessels. Although less common initially, hemorrhages can cause sudden, severe neurological deficits, including loss of consciousness, seizures, or coma.
Early diagnosis of Moyamoya disease is critical in children because timely surgical intervention can significantly reduce the risk of stroke and improve outcomes. Diagnostic imaging, especially cerebral angiography, magnetic resonance angiography (MRA), or computed tomography angiography (CTA), helps visualize the characteristic narrowing of the arteries and the development of abnormal collateral vessels.
Overall, awareness of the symptoms associated with Moyamoya disease in children is essential. Recognizing early warning signs such as transient weakness, speech difficulties, or unexplained neurological changes can prompt prompt medical evaluation, leading to interventions that can prevent devastating strokes and support better neurological health and development.
