Advanced

AJNR - American Journal of Neuroradiology

This Article Abstract Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Mascalchi, M. Articles by Bugiani, O. Search for Related Content PubMed PubMed Citation Articles by Mascalchi, M. Articles by Bugiani, O.

CT and MR Imaging of Neuroaxonal Leukodystrophy Presenting as Early-Onset Frontal Dementia

M. Mascalchi^a, C. Gavazzi^a, M. Morbin^c, G. Giaccone^c, G. Arnetoli^b, R. Zappoli^b and O. Bugiani^c

^a Radiodiagnostic Section, Department of Clinical Physiopathology
^b Department of Neurological Sciences, University of Florence, Florence, Italy
^c Division of Neuropathology, Carlo Besta Neurological Institute, Milan, Italy

View larger version (73K): [in a new window]   Fig 1. Axial contrast-enhanced CT scan (A); unenhanced axial T1-weighted (B) MR image (repetition time, 460 milliseconds; echo time, 20 milliseconds; number of excitations, 4 [TR/TE/NEX]), and T2-weighted (C) MR image (2000/100/2) show low attenuation and signal intensity changes confined to the frontal white matter and the genu of the corpus callosum. No contrast enhancement or overt atrophy is present.

View larger version (56K): [in a new window]   Fig 2. A, Tolouidine blue staining (60x) of the stereotactic biopsy of the right frontal subcortical white matter shows numerous astrocytes and myelinated fibers and an axonal dilation spheroid (arrow). B, Paraffin section of the affected white matter (15x) stained with antibodies to neurofilaments (DAKO) demonstrates intense staining within a spheroid. C, Ultrastructural examination (8000x) of the affected white matter shows a swollen axon whose cytoplasm is filled in the central area with altered mithocondria and attenuated bodies, whereas neurofilaments and neurotubules tend to occupy the periphery.