Cribriform pattern in brain MRI: A diagnostic clue for mucopolysaccharidoses

SA Journal of Radiology

Field Value
Title Cribriform pattern in brain MRI: A diagnostic clue for mucopolysaccharidoses
Creator Biswas, Shamick Chakraborty, Sumit
Subject Radiology cribriform pattern; mucopolysaccharidoses (MPS); perivascular spaces (PVS) Neuroradiology
Description 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.
Publisher AOSIS
Date 2013-11-08
Type info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion — Case report
Format text/html application/pdf
Identifier 10.4102/sajr.v17i4.10
Source South African Journal of Radiology; Vol 17, No 4 (2013); 147-148 2078-6778 1027-202X
Language eng
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Coverage Asia; India; Institute of Post-Graduate Medical Education and Research and Seth Sukhlal Karnani Memorial (IPGME&R and SSKM) Hospital — —
Rights Copyright (c) 2013 Shamick Biswas, Sumit Chakraborty