Central nervous system involvement in systemic mastocytosis (SM) is very rare. This case report describes the computed tomography and magnetic resonance (MR) imaging findings of central nervous system involvement in a patient with isolated bone marrow mastocystosis. Bone marrow infiltration in SM caused cranial nerve dysfunction and meningeal irritation secondary to narrowing of cranial apertures and meningeal involvement, respectively. MR imaging is the modality of choice in both detection and follow-up of SM and also useful for differential diagnosis and detection of complications.
ValentPHornyH-PLiCY: Mastocytosis (mast cell disease). World Health Organization (WHO) classification of tumours. Pathology and genetics. In: JaffeESHarrisNLSteinHVardimanJW, (Eds). Tumours of haematopoietic and lymphoid tissues. Vol 1. World Health Organization. Lyon, France: IARC Press2001: 291–302.
3.
ValentPHornyH-PEscribanoL: Diagnostic criteria and classification of mastocytosis: A consensus proposal. Conference Report of Year 2000 Working Conference on Mastocytosis. Leuk Res25: 603–25, 2001.
4.
ValentPSperrWRSchwartzLB: Diagnosis and classification of mast cell proliferative disorders: Delineation from immunologic diseases and non-mast cell hematopoietic neoplasms. J Allergy Clin Immunol114: 3–11, 2004.
5.
AvilaNALingAMetvalfeDD: Mastocytosis: Magnetic resonance imaging patterns of marrow disease. Skeletal Radiol27: 119–126, 1998.
6.
HoLHLipperM: Mastocytosis of axial skeleton presenting as an epidural mass lesion: MR imaging appearance. Am J Radiol167: 716–718, 1996.
7.
HermannGShapiroRSAbdelwahabIH: MR imaging in adults with Gaucher disease type Y: Evaluation of marrow involvement and disease activity. Skeletal Radiol22: 247–251, 1993.
8.
HarnsbergerHR (Ed): The skull base. In: Handbook of Head and Neck Imaging. 2nd ed, Mosby, St Louis1995: 399–425.
9.
RocaMMotaJGiraldoP: Systemic mastocytosis: MRI of bone marrow involvement. Eur. Radiol9: 1094–1097, 1999.
