Neuroradiology of Pelizaeus-Merzbacher disease: a long-term follow-up study
Pelizaeus-Merzbacher disease (PMD) is a leuko-dystrophy linked to an arrest of myelin formation of unknown aetiology. We report a longitudinal study (1–6 years) of seven affected patients. In all patients, MRI showed diffuse alteration of the supratentorial white matter. The infratentorial white matter was involved in the conatal form as well. Intermediate appearences were seen in the classical and non-X-linked forms. Except for the conatal case, little or no neuroradiological progression was seen in contrast with the slow progressive clinical worsening.
key wordsPalizaeus-Merzbacher disease Magnetic resonance imaging Follow-up
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