Neuropsychiatric Symptoms of Moyamoya Disease: Considerations for the Clinician

Neurocognitive impairment in moyamoya disease is common, under recognized, and potentially devastating. The purpose of this paper is to provide an updated overview on this topic for the practicing clinician. We searched PubMed for keywords including cognitive impairment, neurocognitive dysfunction, and neuropsychological recovery in moyamoya disease. We summarized the literature to provide a concise review of the treatment and management of neuropsychiatric symptoms associated with moyamoya disease. Neuropsychiatric sequelae have conventionally been attributed to chronic cerebral hypoperfusion and/or stroke. Cognitive dysfunction in adults with moyamoya disease is most commonly in the form of impaired executive function, whereas intelligence is the predominant impairment in children with moyamoya disease. Pharmacotherapy for treatment of the neuropsychiatric symptoms associated with moyamoya disease is appropriate and can improve quality of life; however, careful consideration is needed to avoid adverse cerebrovascular events. It remains unclear as to whether surgical revascularization improves or stabilizes cognitive performance and outcomes. Additional prospective studies are warranted to better understand the long-term impact of revascularization on cognitive functioning in moyamoya disease.