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Neuropediatrics 2006; 37(2): 110-113
DOI: 10.1055/s-2006-924226
Short Communication

Georg Thieme Verlag KG Stuttgart · New York

Atypical MRI Findings in Kearns-Sayre Syndrome: T2 Radial Stripes

R. G. Hourani1 , W. M. Barada2 , A. M. Al-Kutoubi1 , M. H. Hourani1
  • 1Department of Diagnostic Radiology, The American University of Beirut Medical Center, Beirut, Lebanon
  • 2Department of Neurology, The American University of Beirut Medical Center, Beirut, Lebanon
Further Information

Publication History

Received: November 4, 2005

Accepted after Revision: April 20, 2006

Publication Date:
14 June 2006 (online)

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Abstract

Kearns-Sayre syndrome (KSS) is a mitochondrial disorder consisting of external ophthalmoplegia, retinitis pigmentosa, ataxia and heart block. Magnetic resonance imaging (MRI) shows abnormal T2 high signal intensity in the deep gray matter nuclei, the cerebellar and the subcortical white matter. We report an unusual MR pattern of KSS, where the T2 images revealed radially oriented, hypointense stripes in hyperintense white matter, a characteristic MRI pattern of lysosomal disease not previously reported in KSS.

Key words

Kearns-Sayre syndrome - MRI - radial stripes

References

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M.D. Roula Hourani

Department of Diagnostic Radiology
The American University of Beirut Medical Center

Riad El Solh 1107 2020

Beirut

P. O. Box 11-0236

Lebanon

Email: rh64@aub.edu.lb