This Article Full Text (PDF) References Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Saved Citations Download to citation manager Request Permissions Request Reprints Add to My Marked Citations Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Citing Articles via Scopus Google Scholar Articles by Vanhanen, S.-L. Articles by Santavuori, P. Search for Related Content PubMed PubMed Citation Articles by Vanhanen, S.-L. Articles by Santavuori, P. Social Bookmarking                         What's this?

MRI Evaluation of the Brain in Infantile Neuronal Ceroid-Lipofuscinosis

Part 2: MRI Findings in 21 Patients

Department of Child Neurology

Raili Raininko, MD

Department of Radiology, University of Helsinki, Helsinki, Finland

Taina Autti, MD

Department of Child Neurology, Department of Radiology, University of Helsinki, Helsinki, Finland

Pirkko Santavuori, MD

Department of Child Neurology

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. (J Child Neurol 1995; 10:444-450).

Journal of Child Neurology, Vol. 10, No. 6, 444-450 (1995)
DOI: 10.1177/088307389501000604


CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				R. Schiffmann and M. S. van der Knaap Invited Article: An MRI-based approach to the diagnosis of white matter disorders Neurology, February 24, 2009; 72(8): 750 - 759. [Abstract] [Full Text] [PDF]

				J. Tyynela, T. Autti, M. Haltia, and S. E. Mole Pirkko Santavuori (1933-2004) J Child Neurol, June 1, 2004; 19(6): 465 - 470. [PDF]

				Z. Lukacs, P. Santavuori, A. Keil, R. Steinfeld, and A. Kohlschutter Rapid and Simple Assay for the Determination of Tripeptidyl Peptidase and Palmitoyl Protein Thioesterase Activities in Dried Blood Spots Clin. Chem., March 1, 2003; 49(3): 509 - 511. [Full Text] [PDF]

				T. Lonnqvist, S. L. Vanhanen, K. Vettenranta, T. Autti, J. Rapola, P. Santavuori, and U.M. Saarinen-Pihkala Hematopoietic stem cell transplantation in infantile neuronal ceroid lipofuscinosis Neurology, October 23, 2001; 57(8): 1411 - 1416. [Abstract] [Full Text] [PDF]

				K. E. Wisniewski, N. Zhong, and M. Philippart Pheno/genotypic correlations of neuronal ceroid lipofuscinoses Neurology, August 28, 2001; 57(4): 576 - 581. [Abstract] [Full Text] [PDF]

				R. Riikonen, S.-L. Vanhanen, J. Tyynela, P. Santavuori, and U. Turpeinen CSF insulin-like growth factor-1 in infantile neuronal ceroid lipofuscinosis Neurology, May 9, 2000; 54(9): 1828 - 1832. [Abstract] [Full Text] [PDF]

				P. Sonninen, T. Autti, T. Varho, M. Hämäläinen, and R. Raininko Brain Involvement in Salla Disease AJNR Am. J. Neuroradiol., March 1, 1999; 20(3): 433 - 443. [Abstract] [Full Text]

				T. Autti, R. Raininko, M. Haltia, L. Lauronen, S.-L. Vanhanen, O. Salonen, H. J. Aronen, K. Wirtavuori, and P. Santavuori Aspartylglucosaminuria: Radiologic Course of the Disease With Histopathologic Correlation J Child Neurol, September 1, 1997; 12(6): 369 - 375. [Abstract] [PDF]

				S.-L. Vanhanen, R. Raininko, P. Santavuori, T. Autti, and M. Haltia MRI Evaluation of the Brain in Infantile Neuronal Ceroid-Lipofuscinosis. Part 1: Postmortem MRI With Histopathologic Correlation J Child Neurol, November 1, 1995; 10(6): 438 - 443. [Abstract] [PDF]