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AJNR - American Journal of Neuroradiology

Published ahead of print on April 3, 2008
doi: 10.3174/ajnr.A1060

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Can MR Imaging Diagnose Adult-Onset Alexander Disease?

L. Farina^a, D. Pareyson^b, L. Minati^a,c, I. Ceccherini^d, L. Chiapparini^a, S. Romano^e, P. Gambaro^f, R. Fancellu^b and M. Savoiardo^a

^a Department of Neuroradiology, Fondazione IRCCS Istituto Neurologico "C. Besta," Milan, Italy
^b Departments of Biochemistry and Genetics, Fondazione IRCCS Istituto Neurologico "C. Besta," Milan, Italy
^c Department of Scientific Direction Unit, Fondazione IRCCS Istituto Neurologico "C. Besta," Milan, Italy
^d Laboratory of Molecular Genetics, Institute G. Gaslini, Genoa, Italy
^e Department of Neurology and Centre for Experimental Neurological Therapy, S. Andrea Hospital, University of Rome, Rome, Italy
^f the Clinica Neurologica L. Sacco Hospital, Milan, Italy

View larger version (116K): [in this window] [in a new window]   Fig 1. A, T2-weighted sagittal section in patient 9 shows thinning of the medulla oblongata and spinal cord with hyperintensity in the medulla; arrow indicates its posterior concave profile. B–D, Axial T2-weighted sections (thickness 3 mm) in the same patient demonstrate increased signal intensity involving the hilum of the dentate nuclei (arrow on the left side), the medial lemniscus (arrowhead, B), and the corticospinal tracts (arrowheads on the left side) in the medulla (C and D). In patient 11 (E), axial spinal cord T2-weighted section at the C1-C2 level shows severe atrophy with mild changes in signal intensity.

View larger version (166K): [in this window] [in a new window]   Fig 2. Axial and coronal FLAIR images in patient 6 show signal intensity changes prevalent in the cerebral posterior periventricular regions (A and B) and involvement of the hilum of the dentate nuclei (arrow on the left side, B). In patient 11, coronal FLAIR image (C) shows a thin bilateral band of periventricular hyperintensity (arrowheads on the right) not recognizable on T2-weighted images (not shown). In patient 3, midbrain peripheral rim of hyperintensity (arrow) is seen only on FLAIR section (D).

View larger version (69K): [in this window] [in a new window]   Fig 3. In patient 3, sagittal T1-weighted section (A) shows severe atrophy of the medulla oblongata (arrow) and spinal cord. Moderate cerebellar and cerebral atrophy is also present. Postcontrast axial sections (B and C) demonstrate 2 small areas of abnormal enhancement (arrows).