Neville Irani MD, Presenter: Nothing to Disclose

Karuna Vithal Shekdar MD, Abstract Co-Author: Nothing to Disclose

Tamara Feygin MD, Abstract Co-Author: Nothing to Disclose

Avrum Nathaniel Pollock MD, Abstract Co-Author: Nothing to Disclose

Robert A. Zimmerman MD, Abstract Co-Author: Nothing to Disclose

1. To illustrate imaging and MR spectroscopy findings of lysosomal storage disorders in the pediatric population. 2. To help the radiologist narrow the diagnostic possibilities for a clinician dealing with a child diagnosed with 'metabolic disorder'.

A didactic format with a combination of text, images, and diagrams will be followed by case presentation in a quiz format to illustrate the key findings using examples. Patterns of anatomic involvement will be emphasized with an algorithmic approach to improve diagnostic classification. Comparison with other metabolic disorders will illustrate characteristic features of lysosomal disorders. A summary table of the clinical findings for common lysosomal storage diseases will be included.

This exhibit will help classify lysosomal storage disorders by prevelance and characteristic neurologic, MR spectroscopy, and imaging findings. We will include examples of the more common lysosomal disorders such as Metachromatic Leukodystrophy, Krabbe’s disease, Mucopolysaccharidoses (Hunter’s and Hurler’s), Gangliosidosis (Tay-Sachs), and Glycogen storage disease. Other classes of metabolic disorders, such as disorders of amino acid metabolism will also be briefly mentioned for comparison.

Irani, N, Shekdar, K, Feygin, T, Pollock, A, Zimmerman, R, Review of Brain and Spine Imaging of Lysosomal Storage Disorders in the Pediatric Patient.  Radiological Society of North America 2010 Scientific Assembly and Annual Meeting, November 28 - December 3, 2010 ,Chicago IL. http://archive.rsna.org/2010/9012863.html