Case Report

Cribriform pattern in brain MRI: A diagnostic clue for mucopolysaccharidoses

Shamick Biswas, Sumit Chakraborty
South African Journal of Radiology | Vol 17, No 4 | a10 | DOI: https://doi.org/10.4102/sajr.v17i4.10 | © 2013 Shamick Biswas, Sumit Chakraborty | This work is licensed under CC Attribution 4.0
Submitted: 21 February 2013 | Published: 08 November 2013

About the author(s)

Shamick Biswas, Department of Radiodiagnosis, Institute of Post-Graduate Medical Education and Research, and Seth Sukhlal Karnani Memorial (IPGME&R and SSKM) Hospital, Kolkata, West Bengal, India
Sumit Chakraborty, Department of Radiodiagnosis, Institute of Post-Graduate Medical Education and Research, and Seth Sukhlal Karnani Memorial (IPGME&R and SSKM) Hospital, Kolkata, West Bengal, India

Abstract

Mucopolysaccharidoses (MPS) represents a heterogeneous group of inherited lysosomal storage disorders characterised by defective degradation of long-chain complex carbohydrates called glycosoaminoglycans (GAGs). To date, 11 distinct types of MPS have been described, each as a result of deficient enzymatic activity of specific lysosomal hydrolase. The most common types are Hurler and Hunter syndromes. We report a case of a child presenting with macrocephaly, clinically suspected to be due to hydrocephalus. An MRI (3 Tesla) brain study demonstrated the cribriform pattern in the brain caused by dilated perivascular spaces, which is a diagnostic clue for the presence of MPS.

Keywords

cribriform pattern; mucopolysaccharidoses (MPS); perivascular spaces (PVS)

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