Case Report: Disturbed Sleep in a Patient with Moyamoya Disease

Background: Moyamoya disease is a chronic, progressive occlusion of the circle of Willis arteries that leads to the development of characteristic collateral vessels. The incidence of moyamoya disease is highest in Japan, with 0.35 cases per 100,000 people. At this time, moyamoya disease is believed to be hereditary and the pathogenesis is unknown.

Case report: This case report describes an 11-year-old Asian girl diagnosed with idiopathic moyamoya disease. The patients have a history of severe migraines and sleep disturbances and experienced a stroke-like event with balance, coordination, sensory and motor function impairment. She was subsequently treated with three revascularisation surgeries. Prior to her presentation, the patient experienced severe migraines attacks 4-5 times per week, with migraines representing a primary concern since the age of 5. She experienced difficulties falling asleep and maintaining sleep and developed mood-related problems. A full montage electroencephalogram (EEG) showed abnormal background activity and revealed epileptiform changes. Following treatment of her sleep disturbance with Tryptophan, her migraines improved and functional abilities were also enhanced. Her improvement in sleep function was quantified via repeated polysomnographic studies and actigraphy monitoring between 1/2012 and 2/2014. Along with the details of this case, this article provides a review of the literature related to this condition.

Conclusion: This case report demonstrates that investigation of sleep-related concerns is able to provide a window into neurologic abnormalities, allowing for enhanced detection of these issues and improved therapeutic outcomes. A review of the literature indicates that little information is available related to the pathogenesis of moyamoya disease and that the presentation of sleep disturbances has not been widely reported. We believe this case report will help to improve understanding of the disease and its presentation.