Erdheim Chester disease (ECD): clinical features

Evidence-based neurology checklist on erdheim chester disease (ecd): clinical features: Neurological features Cerebellar ataxia Cognitive impairment Bulbar affect Chorea Compressive myelopathy Peripheral neuropathy (PN) Seizures Intracranial mass lesions Meningitis with hypoglycorrhachia Ocular…

Neurological features

  • Cerebellar ataxia
  • Cognitive impairment
  • Bulbar affect
  • Chorea
  • Compressive myelopathy
  • Peripheral neuropathy (PN)
  • Seizures
  • Intracranial mass lesions
  • Meningitis with hypoglycorrhachia

Ocular features

Dermatological features

Endocrine features

Haematological features

Otorhinolaryngological features

Cardiac features

Other features

Differential diagnosis

Predictors of poor prognosis

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 27 more. Subscribe to see the full list

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