Characteristics of cognitive impairment in adult asymptomatic moyamoya disease

Abstract

Background: Cognitive impairment in adult moyamoya disease (MMD) is thought to be the result of ischemic stroke; however, the presence and extent of cognitive decline in asymptomatic patients is unclear. Methods: After classification using T2-weighted fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging (MRI), a total of 19 MMD patients with a history of cerebral infarction, 21 asymptomatic MMD patients, and 20 healthy controls matched for age, sex, and years of education were prospectively included in this study. A detailed neuropsychological evaluation of two moyamoya subgroups and normal controls was conducted. Results: Asymptomatic patients showed varying degrees of decline in intelligence (Raven's Standard Progressive Matrices, P = 0.001), spatial imagination (mental rotation, P = 0.014), working memory (verbal working memory-backward digit span, P = 0.011), and computational ability (simple subtraction, P = 0.014; complex subtraction, P < 0.001) compared with normal controls. MMD patients with cerebral infarction had more severe impairment in complex arithmetic (P = 0.027) and word short-term memory (P = 0.01) than those without symptoms. Conclusion: In asymptomatic MMD patients, a variety of cognitive impairment precedes the onset of clinical symptoms such as cerebral infarction, which may be a long-term complication of conservative treatment.

Silent Moyamoya disease - A rare case report

“…Patients typically present with acute cerebrovascular events, including ischemic stroke, transient ischemic attacks (TIA), intracranial hemorrhages, occasionally seizures, and cognitive decline [1 , 2 , 8] . The progressive cerebral hypoperfusion eventually promotes cognitive impairment, intellectual decline or mental retardation [2] .…”

“…Incidental findings of asymptomatic MMD have been reported in several studies [6 , 7 , [9] , [10] , [11] , [12] . Patients with angiographic evidence of MMD without any ischemic or hemorrhagic episodes are classified as asymptomatic or silent MMD [6] , [7] , [8] , [9] .…”

“…Nevertheless, there have been previous reports of asymptomatic cases of MMD, in literature. A well-established definition of asymptomatic MMD has not yet been agreed upon, however most studies define the absence of clinical and imaging findings associated with ischemic or hemorrhagic strokes, in the context of imaging findings suggestive of Moyamoya disease, as asymptomatic MMD [6] , [7] , [8] , [9] . Cognitive impairment affecting intelligence, spatial abilities, verbal working memory and number manipulation is present in asymptomatic patients [8] .…”

“…A well-established definition of asymptomatic MMD has not yet been agreed upon, however most studies define the absence of clinical and imaging findings associated with ischemic or hemorrhagic strokes, in the context of imaging findings suggestive of Moyamoya disease, as asymptomatic MMD [6] , [7] , [8] , [9] . Cognitive impairment affecting intelligence, spatial abilities, verbal working memory and number manipulation is present in asymptomatic patients [8] . Data from several studies shows that asymptomatic MMD is a progressive pathological entity, promoting disturbances in cerebral hemodynamics that eventually lead to ischemic or hemorrhagic strokes [6] , [7] , [8] , [9] .…”

“…Cognitive impairment affecting intelligence, spatial abilities, verbal working memory and number manipulation is present in asymptomatic patients [8] . Data from several studies shows that asymptomatic MMD is a progressive pathological entity, promoting disturbances in cerebral hemodynamics that eventually lead to ischemic or hemorrhagic strokes [6] , [7] , [8] , [9] .…”