ABSTRACT
BACKGROUND: Moyamoya usually presents with cerebral ischemia in children and intracranial hemorrhage in adults. Treatment remains controversial. DESIGN AND OBJECTIVE: We reviewed our experience from June 1995 to August 1999 of 20 adult and pediatric angiographically diagnosed patients with moyamoya to report their clinical presentation, radiological findings, management, and clinical outcomes. RESULTS: The mean age of patients at symptom onset was 17 years (range, 2-54 years). Patients were divided into 2 age groups (group 1, <18 years; group 2, > or =18 years). There were 13 patients in group 1 and 7 patients in group 2. Ischemic strokes or transient ischemic attacks were the predominant initial presentations in both groups. One patient in group 2 had an intraparenchymal brain hemorrhage. Five patients received medical treatment, and 15 had surgical revascularization. The mean time from symptom onset to surgical procedure was significantly longer for patients in group 1 than for those in group 2 (P =.03). The mean follow-up time was 36 months. One patient in group 1 had an ischemic stroke. There was no difference in stroke recurrence, mortality, or modified Rankin scale score among medically or surgically treated patients. CONCLUSIONS: Moyamoya disease may have a different presentation and more benign natural history in our population than in Asian populations. Our findings emphasize the need to better understand the natural history of patients with moyamoya as well as the clinical benefit of different treatment modalities. Structured multicenter randomized clinical trials are needed to further assess the best treatment modalities for patients with moyamoya in the United States.
Subject(s)
Moyamoya Disease/physiopathology , Adolescent , Adult , Age of Onset , Cerebral Revascularization , Child , Child, Preschool , Female , Humans , Male , Middle Aged , Moyamoya Disease/diagnosis , Moyamoya Disease/ethnology , Moyamoya Disease/surgery , Treatment OutcomeABSTRACT
BACKGROUND AND PURPOSE: It is important to adjust stroke outcomes for differences in initial stroke severity. The NIH Stroke Scale (NIHSS) is a commonly used stroke severity measure but has been validated for retrospective scoring only in a subset of stroke clinical trial participants. The purpose of this research was to assess the validity and reliability of an algorithm for retrospective NIHSS scoring in a setting with usual chart documentation. METHODS: An algorithm for retrospective NIHSS scoring was developed with written history and physical admission notes. Missing physical examination data were scored as normal. One investigator prospectively scored the admission NIHSS in 32 consecutive stroke patients. Two raters retrospectively scored the NIHSS by applying the algorithm to photocopied admission notes. Linear regression was used to assess interrater reliability and agreement between prospective and retrospective NIHSS scores. The Wilcoxon signed rank test was used to assess systematic scoring bias. Weighted kappa statistics were calculated to assess the level of agreement of individual NIHSS items. RESULTS: Only 1 admission note was complete for all NIHSS elements. Interrater reliability was near perfect (r(2)=0.98, P<0. 001). Agreement between prospective and retrospective NIHSS score was also excellent (r(2)=0.94, P<0.001) and there was no systematic bias in retrospective scores. Agreement for individual items was moderate to high for all items except level of consciousness. CONCLUSIONS: Retrospective NIHSS scoring with the algorithm is reliable and unbiased even when physical examination elements are missing from the written record. Stroke research using retrospective review of charts or of administrative databases should adjust for differences in stroke severity using such an algorithm.
