Clinical presentation, progression, and treatment outcomes of moyamoya disease in the elderly.

Background: Moyamoya disease is a vascular disorder characterized by progressive stenosis of the internal carotid artery. The presentation, progression, treatment options, and post-operative clinical outcomes for elderly (60 and older) Moyamoya patients have never been reported.

Methods: A retrospective analysis of all patients who were diagnosed with Moyamoya disease by the senior authors between 1991 and 2016 was performed. Patients who were 60 years or older at the time of surgery or last follow-up were further evaluated.

Results: Seventy patients were diagnosed with probable or definite Moyamoya disease during the study period (1991-2016). Eight patients (11.4 %; six females: two males; median age 63; range, 60-71 years) were found to be 60 years or older at the time of surgery or last follow-up and were included in the study. All patients had a modified Rankin scale (mRS) of either one or two (median 1) pre-operatively. Six patients (75 %) underwent surgical treatment on a total on seven hemispheres. Post-surgery, one patient had an improved mRS score, three had no changes, and two had worsening in their mRS scores. Both patients who did not undergo surgical interventions suffered from intra-parenchymal hemorrhages post-diagnosis.

Conclusions: Moyamoya disease is most commonly seen in young and middle-aged patients. Presentation in the elderly (defined as 60 years and older in this study) is rare, and has never been reported in the literature. In this study, both direct and indirect revascularization procedures demonstrated potential benefit in some of these patients, with stabilization of progressive symptoms.