Early-onset dementia, leukoencephalopathy and brain calcifications: a clinical, imaging and pathological comparison of ALSP and PLOSL/Nasu Hakola disease

@article{Coomans2018EarlyonsetDL,
  title={Early-onset dementia, leukoencephalopathy and brain calcifications: a clinical, imaging and pathological comparison of ALSP and PLOSL/Nasu Hakola disease},
  author={C Coomans and Anne Sieben and Martin Lammens and Chantal Ceuterick-de Groote and Caroline Vandenbroecke and Ingeborg Goethals and Deborah Van Melkebeke and Dimitri Hemelsoet},
  journal={Acta Neurologica Belgica},
  year={2018},
  volume={118},
  pages={607-615}
}
Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia, and Nasu Hakola disease or polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy are both underrecognized progressive degenerative white matter diseases that can present with young dementia, leukoencephalopathy and brain calcifications. We report and compare three cases in terms of clinical phenotype, imaging and neuropathological findings. Both cases have led to the identification of two novel… CONTINUE READING

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