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Moyamoya disease with a marked collateral supply through the artery of Bernasconi-Cassinari
I Pascual-Castroviejo1, J Viano, S I Pascual-Pascual
1Service of Pediatric Neurology and Autonoma University, Hospital Infantil 'La Paz', Madrid, Spain.
Insights
This case study highlights moyamoya disease in a young girl, showing early onset and symptom improvement with nicardipine. Magnetic resonance arteriography (MRA) proved superior for detecting intracranial vessel anomalies.
Area of Science:
- Neurology
- Vascular Neurology
- Pediatric Neurology
Background:
- Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by stenosis of the terminal portion of the internal carotid arteries and the arteries of the circle of Willis.
- Early diagnosis and intervention are crucial for managing neurological symptoms such as seizures and transient ischemic attacks.
Observation:
- A 15-year-old female presented with a history of seizures, transient ischemic attacks, and left hemiparesis since 8 months of age.
- Cerebral arteriography at 1 year revealed moyamoya disease features; a rare embryonic tentorial artery (Bernasconi-Cassinari artery) was noted.
- This artery was absent at 6 years but reappeared on magnetic resonance arteriography (MRA) at 15 years.
Findings:
- The fluctuating presence of the Bernasconi-Cassinari artery suggests a very early intrauterine onset of moyamoya disease.
- Magnetic resonance arteriography (MRA) demonstrated superior capability in detecting intracranial vessel anomalies compared to conventional arteriography.
- Nicardipine, a calcium channel blocker, combined with antiepileptic drugs, improved both epileptic and neurological symptoms.
Implications:
- This case underscores the potential for intrauterine onset of moyamoya disease and the diagnostic advantage of MRA in identifying complex vascular anomalies.
- The findings support the use of calcium channel blockers like nicardipine as an adjunct therapy for managing neurological deficits in moyamoya disease.
- Further research into the early developmental aspects and advanced imaging techniques for moyamoya disease is warranted.
Abstract:
A case of moyamoya disease is presented. The patient is a 15-year-old white Spanish girl who had the onset of the neurological symptoms at 8 months of age with seizures, transient ischemic attacks and residual left hemiparesis. Cerebral arteriography performed at the age of 1 year revealed all the features of moyamoya disease in the territory of both carotids and in the vertebrobasilar arteries. The presence of an embryonic tentorial artery, the Bernasconi-Cassinari artery, originating from the right internal carotid artery, was disclosed at 1 year of age but did not appear in an arteriogram performed at 6 years of age and it was revisualized by magnetic resonance arteriography (MRA) performed at the age of 15 years. This finding seems to indicate a very early intrauterine onset of the disease in this case and demonstrates the superiority of MRA over conventional arteriography to discover anomalies of intracranial vessels. Administration of nicardipine, a calcium channel blocker, added to conventional antiepileptic drugs that the patient had previously taken, improved the epileptic and the neurological disease.