0
June 1977, Vol 131, No. 6 >
Article | June 1977

Kearns-Sayre Syndrome: The Importance of Early Recognition FREE

ARTHUR M. SEIGEL, MD; BENNETT A. SHAYWITZ, MD; THOMAS CIESIELSKI, MD
Am J Dis Child. 1977;131(6):711-712. doi:10.1001/archpedi.1977.02120190105025.
Text Size: A A A
Published online
Article
References
Comments

Progressive external ophthalmoplegia, retinitis pigmentosa, and heart block (Kearns-Sayre syndrome) is a rare disorder that most commonly begins in childhood, with ptosis as the initial sign.1 The syndrome has not been presented before in the pediatric literature, and all previous cases of the disorder have been reported in the adult neurological or ophthalmological literature where debate has centered mainly about the pathogenesis of the disorder. It has been emphasized recently that, with the advent of pacemaker treatment of heart block, early recognition of the syndrome has taken on potentially therapeutic significance.2

This case report is of a young woman who has been observed in the Pediatric Neurology Clinic at the Yale-New Haven Hospital since the age of 11 years with the characteristic signs of the Kearns-Sayre syndrome, and who also has juvenile diabetes mellitus. The latter has not previously been reported with this syndrome and its significance is

REFERENCES

1 +
Kearns TP, Sayre GP:  Retinitis pigmentosa, external ophthalmoplegia, and complete heart block . Arch Ophthal 60:280, 1958;.
Link to Article
2 +
Ross A, Lipschutz D, Austin J, et al:  External ophthalmoplegia and complete heart block . N Engl J Med 280:313-314, 1969;.
Link to Article
3 +
Drachman DA:  Ophthalmoplegia plus . Arch Neurol 18:654-674, 1968;.
Link to Article
4 +
Olson W, Engel WK, Walsh GO, et al:  Oculocraniosomatic neuromuscular disease with ragged-red fibers . Arch Neurol 26:193-211, 1972;.
Link to Article
5 +
Karpati G, Carpenter S, Larbrisseau A, et al:  The Kearns-Sayre syndrome . J Neurol Sci 19:133-151, 1973;.
Link to Article
6 +
Carroll JE, Zwillich C, Weil JV, et al:  Abnormal ventilatory drive in the ragged-red fiber syndrome . Neurology 25:348, 1975;.

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

References

1 +
Kearns TP, Sayre GP:  Retinitis pigmentosa, external ophthalmoplegia, and complete heart block . Arch Ophthal 60:280, 1958;.
Link to Article
2 +
Ross A, Lipschutz D, Austin J, et al:  External ophthalmoplegia and complete heart block . N Engl J Med 280:313-314, 1969;.
Link to Article
3 +
Drachman DA:  Ophthalmoplegia plus . Arch Neurol 18:654-674, 1968;.
Link to Article
4 +
Olson W, Engel WK, Walsh GO, et al:  Oculocraniosomatic neuromuscular disease with ragged-red fibers . Arch Neurol 26:193-211, 1972;.
Link to Article
5 +
Karpati G, Carpenter S, Larbrisseau A, et al:  The Kearns-Sayre syndrome . J Neurol Sci 19:133-151, 1973;.
Link to Article
6 +
Carroll JE, Zwillich C, Weil JV, et al:  Abnormal ventilatory drive in the ragged-red fiber syndrome . Neurology 25:348, 1975;.

Correspondence

Submit a Letter
CME
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
Clear Answers | Save For Later
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s “Cited By” API will populate this tab (http://www.crossref.org/citedby.html).
Compression-Only CPR: Pushing the Science Forward
Cone
AAM 2010;304:1493-1495.
All you need to read in the other general journals
BMJ 2010;341:c5893-c1494.
Submit a Comment

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

© 2013 American Medical Association. All Rights Reserved.
Powered bySilverchair Information Systems