Acta Neurochirurgica

, Volume 152, Issue 9, pp 1619–1621 | Cite as

Meningioma-like lesions in Erdheim Chester disease

  • Rohana Naqi
  • Muhammad Azeemuddin
  • Romana Idrees
  • Mohammad WasayEmail author
Letter to the editor

Dear Sir,

We are reporting a woman with bilateral, multiple, extra-axial-enhancing lesions mimicking meningiomas. Histopathology of lesions revealed the diagnosis of Erdheim Chester disease (ECD). This report provides clinical and neuroimaging findings of this disease and clues for presurgical diagnosis of ECD.

Case report

A 55-year-old female patient, a hypertensive, was admitted with generalized tonic clonic seizures, fever, cough, and drowsiness. Her symptoms had started 1 month back. Detailed work-up was done, and she was diagnosed to have diabetes insipidus. Her laboratory investigations showed hypernatremia with serum sodium level of 170, WBC count was 21.2, ESR was 94, and C-reactive protein was 20.5. Her chest X-ray showed osteopenia of visualized bone with patchy bony density at lateral ends of both clavicles. Radionuclide skeletal scintigraphy revealed diffusely increased tracer uptake over appendicular skeleton and left hemipelvis, with evidence of bony expansion and photon...


Meningioma Diabetes Insipidus Esomeprazole Desmopressin Histiocytosis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.



Authors are grateful to Prof. Ather Enam FRCS, MD, PhD, Head of Neurosurgery at Aga Khan University for his contribution.


  1. 1.
    Babu RP, Lansen TA, Chadburn A, Kasoff SS (1997) Erdheim–Chester disease of the central nervous system: report of two cases. J Neurosurg 86(5):888–892PubMedCrossRefGoogle Scholar
  2. 2.
    Bianco F, Iacovelli E, Tinelli E, Locuratolo N, Pauri F, Fattapposta F (2009) Characteristic brain MRI appearance of Erdheim–Chester disease. Neurology 73(24):2120–2122PubMedCrossRefGoogle Scholar
  3. 3.
    Johnson MD, Aulino JP, Jagasia M, Mawn LA (2004) Erdheim–Chester disease mimicking multiple meningiomas syndrome. AJNR Am J Neuroradiol 25:134–137PubMedGoogle Scholar
  4. 4.
    Klieger MR, Schultz E, Elkowitz DE (2002) Erdheim–Chester disease: a unique presentation with multiple osteolytic lesions of the spine and pelvis that spared the appendicular skeleton. AJR Am J Roentgenol 178(2):429–432, 5PubMedGoogle Scholar
  5. 5.
    Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D, Wechsler J, Brun B, Remy M et al (1996) Erdheim–Chester disease: clinical and radiologic characteristics of 59 cases. Medicine 75(3):157–169PubMedCrossRefGoogle Scholar
  6. 6.
    Wright RA, Hermann RC, Parisi JE (1999) Neurological manifestations of Erdheim–Chester disease. J Neurol Neurosurg Psychiatry 66(1):72–75PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Rohana Naqi
    • 1
  • Muhammad Azeemuddin
    • 1
  • Romana Idrees
    • 2
  • Mohammad Wasay
    • 3
    Email author
  1. 1.Department of RadiologyAga Khan UniversityKarachiPakistan
  2. 2.Department of PathologyAga Khan UniversityKarachiPakistan
  3. 3.Department of Medicine/NeurologyThe Aga Khan UniversityKarachiPakistan

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