The longitudinal observation of progressive cervicomedullary atrophy, which is characteristic of the adult AxD [4, 5], is interesting. It has been speculated that brainstem atrophy in the adult form of AxD might result from tissue damage related to the contrast enhanced nodular and/or swollen lesions usually observed in the juvenile form of the disease. A recent report of a patient with progressive medullary atrophy has reinforced this hypothesis [11]. The 20-year-old patient had an expanded cervicomedullary lesion with patchy contrast enhancement; progressive atrophy was observed 5.5 years later [11].