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MRI evaluation of the brain in infantile neuronal ceroid-lipofuscinosis: Part 2: MRI findings in 21 patients
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Oncology, Radiology and Clinical Immunology. (RAD)
1995 (English)In: Journal of Child Neurology, ISSN 0883-0738, Vol. 10, no 6, 444-50 p.Article in journal (Refereed) Published
Abstract [en]

The purpose of this study was to demonstrate the course of infantile neuronal ceroid-lipofuscinosis with brain magnetic resonance imaging (MRI) in children aged 3 months to 11 years. Twenty-one patients and 46 neurologically normal controls of the same age were examined. The images were evaluated visually; then signal intensities were measured and related to those of references. MRI abnormalities were detectable before clinical symptoms. The radiologic picture of the brain varied with the duration of the disease. Pathognomonic MRI findings in the early stage of the disease were generalized cerebral atrophy, strong thalamic hypointensity to the white matter and to the basal ganglia, and thin periventricular high-signal rims from 13 months onward on T2-weighted images. In patients over 4 years old, cerebral atrophy was extreme, and the signal intensity of the entire white matter was higher than that of the gray matter, which is the reverse of normal. This study showed that the abnormalities seen on MRI progress rapidly during the first 4 years of life, then stabilize, in conformity with the clinical and histopathologic pictures of infantile neuronal ceroid-lipofuscinosis.

Place, publisher, year, edition, pages
1995. Vol. 10, no 6, 444-50 p.
National Category
Medical and Health Sciences
URN: urn:nbn:se:uu:diva-57553DOI: 10.1177/088307389501000604PubMedID: 8576553OAI: oai:DiVA.org:uu-57553DiVA: diva2:85462
Available from: 2008-10-17 Created: 2008-10-17 Last updated: 2010-02-05Bibliographically approved

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