We report the presence of major cerebral migrational defects in five severely, multiply handicapped children with congenital cytomegalovirus (CMV) infection. These patients had both computed tomographic (CT) scan and magnetic resonance imaging (MRI) evidence of marked migrational central nervous system defects consistent anatomically with the spectrum of lissencephaly-pachygyria, a disorder commonly idiopathic or associated with chromosomal abnormalities or with unknown early gestational insults. Neuroradiologic features included broad, flat gyri, shallow sulci, incomplete opercularization, ventriculomegaly, periventricular calcifications, and white-matter hypodensity on CT scans or increased signal intensity on long-TR MRI scans. Evidence for congenital CMV infection included prenatal onset of microcephaly, periventricular calcifications, neonatal jaundice, hepatomegaly, elevated CMV-specific immunoglobulin M, or viral isolation from urine. Previous reports of the neurologic sequelae of CMV have emphasized varying degrees of psychomotor retardation, cerebral palsy and epilepsy due to polymicrogyria, periventricular calcification, microcephaly, or rarely, hydrocephalus. Our patients appear to represent extremely severe examples of the effects of CMV on neurologic growth, maturation, and development. Recognition of these severe migrational abnormalities was improved by use of MRI, a technique that affords superior definition of the nature and extent of gyral and white-matter abnormalities. We suggest that these abnormalities may be more common than has previously been recognized.

A case of fibrolamellar hepatocellular carcinoma (FL-HCC) associated with adjacent focal nodular hyperplasia (FNH) is described. These two regions were adjacent but distinct, both on gross and microscopic examination. Currently, it is unclear whether FL-HCC rarely arises in preexisting FNH, or whether FNH is a typical response to this vascular variant of hepatocellular carcinoma (HCC). The FNH region, which is peripheral, may be biopsied to exclude the underlying carcinoma, and thus lead to inadequate therapy. Previous reports of this association are reviewed. importance of this observation in terms of patient management and proposed pathogenesis is discussed. Case ReportA 19-year-old woman with a 3-year history of oral contraceptive use (type unknown) was admitted for liver mass evaluation. One and one half months before she was admitted, sharp, episodic, left-sided chest pain developed. Hepatomegaly without splenomegaly was detected at evaluation. A computed tomography (CT) scan showed a hepatic mass. She was transferred to the Hospital of the University of Pennsylvania for further examination. The results of the physical examination were normal except for an 1 I-cm liver (by percussion), which was nontender but palpable 5.0 cm below the right costal margin. Complete blood count and serum electrolytes were normal, as were the alanine and aspartate aminotransferases and gamma glutamyl transpeptidase. Serum alkaline phosphatase was minimally elevated at 256 IU/L (compressed normal hepatic parenchyma < 140 IU/L). Serum alpha-fetoprotein and carcinoembryonic antigen were less than 10 ng/ml and 3 ng/ml, respectively. A nonenhanced and contrast-enhanced abdominal CT scan showed a large, relatively hypodense mass in the right hepatic lobe, with an enhancing central septate region. Selective right hepatic arteriography demonstrated a large hypervascular mass in the inferior portion of the right lobe, with a peripheral arterial supply radially penetrating into the center of the lesion (Figure 1). The parenchymal phase showed a dense capillary blush with a stellate septate pattern and central lucency compatible with a central scar. Vascular encasement. portal vein thrombosis, or arteriovenous shunting were not detected. The lesion was considered consistent with FNH.The patient was operated on and the anterior segment of the right hepatic lobe was removed and sent for pathologic con- 3049

BACKGROUND AND PURPOSE: Meningeal inflammation is implicated in cortical demyelination and disability progression in multiple sclerosis (MS). Gadolinium (Gd)-enhanced 3-dimensional (3D) FLAIR (fluid-attenuated inversion recovery) magnetic resonance imaging (MRI) can identify leptomeningeal enhancement (LME) in MS. Further characterization is needed to determine if LME is an imaging biomarker for meningeal inflammation. We sought to characterize the natural history of LME in the community setting, including persistence/resolution, effect of disease-modifying therapy, scanner variability, timing of acquisition, and imaging pitfalls that may lead to misinterpretation. METHODS: A total of 341 MRI exams with Gd-enhanced 3D-FLAIR were reviewed in MS and non-MS patients to determine frequency of enhancement by MS subtype and association with therapy. A phantom was used to assess scanner variability. Two MS patients with seven LME were imaged at four postinjection time points to generate time-intensity curves. Imaging pitfalls were compiled. RESULTS: A total of 16.6% (40/241) of MS patients revealed LME compared to 8% (8/100) in non-MS patients (P = .04). There was no association with MS subtype, therapy, or disease activity. Detection using General Electric's version of 3D-FLAIR (29%) was greater than with Siemen's 3D-FLAIR (12%) at 1.5T (Tesla) (P < .001). Lesions were generally stable but resolved in 2 patients following high-dose steroids. LME kinetics were heterogeneous, even within patients, without uniform optimal time for acquisition. Enhancement curves exhibited three different variations, similar to the two-compartment model. Imaging pitfalls included enhancements of uncertain biologic significance, cortical veins and anatomic structures, and imaging artifacts.

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