Download PDF
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)

    Permissions and Reprints

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

  • 1 Barker P B, Horská A. Neuroimaging in leukodystrophies.  J Child Neurol. 2004;  19 559-570
    CrossrefPubMedGoogle Scholar
  • 2 Barkovich A J, Good W V, Koch T K, Berg B O. Mitochondrial disorders: analysis of their clinical and imaging characteristics.  AJNR Am J Neuroradiol. 1993;  14 1119-1137
    PubMedGoogle Scholar
  • 3 Barragan-Campos H M, Vallee J N, Lo D. et al . Brain magnetic resonance imaging findings in patients with mitochondrial cytopathies.  Arch Neurol. 2005;  62 737-742
    CrossrefPubMedGoogle Scholar
  • 4 Becker L E. Lysosomes, peroxisomes and mitochondria: function and disorder.  AJNR Am J Neuroradiol. 1992;  13 609-620
    PubMedGoogle Scholar
  • 5 Kendall B E. Disorders of lysosomes, peroxisomes, and mitochondria.  AJNR Am J Neuroradiol. 1992;  13 621-653
    PubMedGoogle Scholar
  • 6 Osborn A G. Diagnostic Neuroradiology. St Louis, MO; Mosby-Year Book 1994: 740-743
    Google Scholar
  • 7 Sacher M, Fatterpekar G M, Edelstein S, Sansaricq C, Naidich T P. MRI findings in an atypical case of Kearns-Sayre syndrome: a case report.  Neuroradiology. 2005;  47 241-244
    CrossrefPubMedGoogle Scholar
  • 8 Simaan E M, Mikati M A, Touma E H, Rötig A. Unusual presentation of Kearns-Sayre syndrome in early childhood.  Pediatr Neurol. 1999;  21 830-831
    CrossrefPubMedGoogle Scholar
  • 9 Valanne L, Ketonen L, Majander A, Suomalainen A, Pihko H. Neuroradiologic findings in children with mitochondrial disorders.  AJNR Am J Neuroradiol. 1998;  19 369-377
    PubMedGoogle Scholar
  • 10 van der Knaap M S, Breiter S N, Naidu S, Hart A A, Valk J. Defining and categorizing leukoencephalopathies of unknown origin: MR imaging approach.  Radiology. 1999;  213 121-133
    CrossrefPubMedGoogle Scholar
  • 11 van der Knaap M S. Magnetic resonance in childhood white-matter disorders.  Dev Med Child Neurol. 2001;  43 705-712
    CrossrefPubMedGoogle Scholar
  • 12 van der Voorn J P, Pouwels P J, Kamphorst W. et al . Histopathologic correlates of radial stripes on MR images in lysosomal storage disorders.  AJNR Am J Neuroradiol. 2005;  26 442-446
    PubMedGoogle Scholar

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

      >