Fabry's disease (FD) is a rare, sex-linked disorder resulting from a-galactosidase deficiency. Cerebrovascular complications have been reported in the literature but have not been systematically analyzed. We report 2 patients and review 51 previously reported cases (descriptive meta-analysis) to clarify the clinical, radiologic, and pathologic features. The average age at onset of cerebrovascular symptoms was 33.8 years for hemizygous individuals (n = 43) and 40.3 years of heterozygotes (n = 10). The most frequent symptoms and signs were as follows (in descending order of frequency): hemiparesis, vertigo/dizziness, diplopia, dysarthria, nystagmus, nausea/vomiting, head pain, hemiataxia, and ataxia of gait, in the hemizygote group; and memory loss, dizziness, ataxia, hemiparesis, loss of consciousness and hemisensory symptoms, in the heterozygote group. The vertebrobasilar circulation was symptomatic in 67% of the hemizygotes and 60% of the heterozygotes. Intracerebral hemorrhage was found in 4 patients (3 hemizygotes and 1 heterozygote). Elongated, ectatic, tortuous vertebral and basilar arteries were the most common angiographic and pathologic features. For the hemizygotes, the recurrence rate for cerebrovascular disease was 76% and the death rate was 55%; 86% of the heterozygotes had recurrent cerebrovascular event(s) and 40% died. The cerebrovascular manifestations of FD, in both hemizygotes and heterozygotes, are predominantly due to dilative arteriopathy of the vertebrobasilar circulation, frequently recur, and portend a poor prognosis. Mitsias P, Levine SR. Cerebrovascular complications of Fabry's disease. Ann Neurol 1996;40:8-17 Patients and Methods W e reviewed our clinical experience with 2 siblings, 1 male hemizygote and 1 female heterozygote, with cerebrovascular manifestations of FD, and systematically reviewed the literature. Criteria for diagnosis of FD and inclusion into this study included the following: corneal abnormalities, angiokeratoma, positive family history, and decreased a-galactosidase activity on cultured skin fibroblasts or platelets. Literature review cases were identified by using a computerized English language Medline search (key words: Fabry's disease,

Background and Purpose-The purpose of this study was to show that the computer segmentation algorithm Iterative Self-Organizing Data Analysis Technique (ISODATA), which integrates multiple MRI parameters (diffusion-weighted imaging [DWI], T2-weighted imaging [T2WI], and T1-weighted imaging [T1WI]) into a single composite image, is capable of defining the ischemic lesion in a time-independent manner equally as well as the MRI techniques considered the best for each phase after stroke onset (ie, perfusion weighted imaging [PWI] and DWI for the acute phase and T2WI for the outcome phase). Methods-We measured MRI parameters of PWI, DWI, T2WI, and T1WI from patients at the acute phase (Ͻ30 hours) and DWI, T2WI, and T1WI at the outcome phase (3 months) of ischemic stroke. The clinical neurological deficit was graded with the National Institutes of Health Stroke Scale (NIHSS). We compared the ISODATA lesion size with the PWI, DWI, and T2WI lesion sizes measured within the same slice at each phase. The lesion sizes were also correlated with NIHSS score of each phase. Results-We included 11 patients; 9 (82%) were women, and 7 (64%) were black. The meanϮSD age was 65.5Ϯ9.3 years (range, 45 to 82 years). The median NIHSS score was 15 (minimum, 4; maximum, 24)at the acute phase and 3 (minimum, 0; maximum, 22) at the outcome phase. The median time interval from stroke symptom onset to the acute MRI study was 10 hours (range, 6 to 29 hours), and the mean time interval to the outcome study was 93Ϯ11 days (range, 72 to 106 days). In the acute phase, the ISODATA lesion size had high correlation with the PWI lesion size (rϭ0.95; 95% CI, 0.89 to 0.98; PϽ0.0001), DWI lesion size (rϭ0.83; 95% CI, 0.66 to 0.92; PϽ0.0001), and T2WI lesion size (rϭ0.67; 95% CI, 0.39 to 0.84; Pϭ0.008) and moderate correlation with NIHSS score (rϭ0.59; 95% CI, 0.02 to 0.88; Pϭ0.06). In the outcome phase, the ISODATA lesion size had high correlation with the T2WI lesion size (rϭ0.97; 95% CI, 0.94 to 0.99; PϽ0.0001) and NIHSS score (rϭ0.78; 95% CI, 0.34 to 0.94; Pϭ0.004). Conclusions-The integrated ISODATA method can identify and characterize the ischemic lesion independently of time elapsed since stroke onset. The ISODATA lesion size highly correlates with the PWI and DWI lesion size in the acute phase and with the T2WI lesion size in the outcome phase of ischemic stroke, as well as with the clinical neurological status of the patient.

tized during the global crisis. It will also guide public health guidelines for at-risk populations to reduce risks of complications from such comorbidities.

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