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Neuroradiology |
1 From the Departments of Radiology (R.v.d.B., M.A.v.B.), Clinical Genetics (S.A.J.L.O.), Pathology (S.G.v.D.), and Neurology (M.B., M.D.F.), Leiden University Medical Center, Albinusdreef 2, C2S, 2333 ZA Leiden, the Netherlands; and Department of Neurology, Rijnland Hospital, Leiderdorp, the Netherlands (J.H.). From the 2001 RSNA scientific assembly. Received June 28, 2001; revision requested August 16; revision received October 29; accepted December 11. Address correspondence to R.v.d.B. (e-mail: r.van_den_boom@lumc.nl).
PURPOSE: To assess the prevalence and distribution of subcortical lacunar lesions (SLLs) in patients with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL), to determine whether SLLs are an abnormal finding by studying their prevalence in healthy subjects, and to assess whether SLLs occur in other conditions associated with small vessel disease and white matter areas of high signal intensity (WMH).
MATERIALS AND METHODS: The presence of SLLs, their location, and their relation to other abnormalities were assessed on magnetic resonance (MR) images (T1-weighted, T2-weighted, and fluid-attenuated inversion-recovery) obtained in 34 CADASIL patients and 20 healthy family members. Three additional control groups of healthy volunteers, elderly patients with vascular risk factors, and patients with another hereditary small vessel disease were also screened for the presence and location of SLLs. Sensitivity and specificity of the presence of SLLs for the diagnosis of CADASIL were assessed.
RESULTS: SLLs were found in 20 (59%) of CADASIL patients. Incidence of SLLs increased with age (20%, <30 years; 50%, 3050 years; 80%, >50 years). SLLs invariably occurred in the anterior temporal lobes and in areas where diffuse WMH expanded into arcuate fibers. From the anterior temporal lobe, the lesions could extend dorsally into the temporal lobes and rostrally into the frontal lobes. Lesions were not found in the parietal and occipital lobes. None of the control subjects had SLLs. Specificity and sensitivity of SLLs for CADASIL were 100% and 59%, respectively.
CONCLUSION: SLLs are an abnormal finding at MR imaging that frequently occur in CADASIL patients.
© RSNA, 2002
Index terms: Brain, diseases, 13.87 Brain, MR, 13.12141 Brain, white matter, 13.87
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