Erdheim Chester disease (ECD): management
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Erdheim Chester disease (ECD): management. Multifocal FLAIR hyperintensities, Nodular enhancement, Cerebral atrophy. Fully referenced neurology checklist.
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
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Other investigations
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Treatment: targeted treatments
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Treatment: conventional agents
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Treatment: others
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Acronym
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References
- 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.
- 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).
- Haroche J, Cohen-Aubart F, Amoura Z. Erdheim-Chester disease. Blood 2020; 135:1311-1318.
- 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).
- Boyd LC, O'Brien KJ, Ozkaya N, et al. Neurological manifestations of Erdheim-Chester Disease. Ann Clin Transl Neurol 2020; 7:497-506.
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