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Absence epilepsy associated with moyamoya disease. Case report
Ken-ichiro Kikuta1, Yasushi Takagi, Yoshiki Arakawa
1Department of Neurosurgery, Kyoto University Graduate School of Medicine, Japan. kikuta@kuhp.kyoto-u.ac.jp
Journal of Neurosurgery
|April 20, 2006
Summary
A child with absence epilepsy and moyamoya disease (MMD) found relief after revascularization surgery. This case highlights MMD as a potential cause for intractable childhood epilepsy requiring surgical consideration.
Area of Science:
- Neurology
- Pediatric Neurology
- Neurosurgery
Background:
- Absence epilepsy is a common epilepsy syndrome in children.
- Moyamoya disease (MMD) is a rare cerebrovascular disorder characterized by progressive stenosis of the internal carotid arteries.
- Hyperventilation can trigger absence seizures in susceptible individuals.
Observation:
- A 6-year-old girl presented with intractable absence epilepsy, characterized by diffuse 3-Hz spike-and-wave complexes on EEG, induced by hyperventilation.
- The patient had underlying moyamoya disease with significant bilateral frontal lobe ischemia.
- Medical management of her epilepsy proved ineffective.
Findings:
- Superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis with encephalomyosynangiosis was performed bilaterally.
- Post-operatively, the patient's epilepsy resolved completely.
- Resolution of ischemic changes in the frontal lobes was observed.
Implications:
- This case suggests moyamoya disease should be considered in the differential diagnosis of children with intractable absence epilepsy.
- Revascularization surgery may be a viable treatment option for epilepsy associated with MMD.
- Early diagnosis and surgical intervention can lead to significant improvement in neurological function and seizure control in pediatric patients with MMD and epilepsy.
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