Summary
Of nine patients with subacute necrotizing encephalomyelopathy, six had cytochrome-c-oxidase (COX) deficiency. Four of these had very similar distribution of signal abnormalities in brain stem and subthalamic nuclei, without putaminal involvement. The putamen was affected in the other two cases of COX deficiency. White matter of cerebellar or cerebral hemispheres was also affected in COX or other enzyme deficiencies, mimicking a leukodystrophy in one case. Even in patients with the same enzymatic defect the pattern of MRI abnormalities is not homogeneous.
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© 1991 Springer-Verlag
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Savoiardo, M., Uziel, G., Strada, L., Visciani, A., Grisoli, M., Wang, G. (1991). MRI findings in Leigh’s disease with cytochrome-c-oxidase deficiency. In: du Boulay, G., Molyneux, A., Moseley, I. (eds) Proceedings of the XIV Symposium Neuroradiologicum. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-49329-4_187
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DOI: https://doi.org/10.1007/978-3-642-49329-4_187
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