Erdheim Chester disease (ECD): management

Evidence-based neurology checklist on erdheim chester disease (ecd): management: Magnetic resonance imaging (MRI) brain: features Multifocal FLAIR hyperintensities Nodular enhancement Cerebral atrophy Demyelination Thick dura mater Spinal dural involvement Meningioma-like tumours Longitudinally…

Magnetic resonance imaging (MRI) brain: features

  • Multifocal FLAIR hyperintensities
  • Nodular enhancement
  • Cerebral atrophy
  • Demyelination
  • Thick dura mater
  • Spinal dural involvement
  • Meningioma-like tumours
  • Longitudinally extensive spinal cord lesions
  • Hypothalamic–pituitary involvement
  • Dentate nucleus hyperintensities
  • Middle cerebellar peduncle hyperintensities

CT scan

Other investigations

Treatment: targeted treatments

Treatment: conventional agents

Treatment: others

Acronym

References

  1. de la Fuente MI, Rosenblum MK, Diamond EL, Tabar VS, Omuro A. Erdheim-Chester disease among neuroinflammatory syndromes: the case for precision medicine. Neurol Neuroimmunol Neuroinflamm 2020; pii: e686.
  2. Bhatia A, Hatzoglou V, Ulaner G, et al. Neurologic and oncologic features of Erdheim-Chester disease: a 30-patient series. Neuro Oncol 2020; pii: noaa008 (Epub ahead of print).
  3. Haroche J, Cohen-Aubart F, Amoura Z. Erdheim-Chester disease. Blood 2020; 135:1311-1318.
  4. Goyal G, Heaney ML, Collin M, et al. Erdheim-Chester disease: consensus recommendations for the evaluation, diagnosis, and treatment in the molecular era. Blood 2020 (Epub ahead of print).
  5. Boyd LC, O'Brien KJ, Ozkaya N, et al. Neurological manifestations of Erdheim-Chester Disease. Ann Clin Transl Neurol 2020; 7:497-506.
  6. And 8 more. Subscribe to see the full list

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