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[Kearns' syndrome (author's transl)]

J Haas, P Haller, U Patzold

Deutsche Medizinische Wochenschrift (1946)

|October 15, 1976

Summary

This summary is machine-generated.

Kearns-Sayre syndrome is a rare condition causing chronic progressive external ophthalmoplegia, retinal changes, and cardiac defects. Early pacemaker implantation can prevent severe cardiac events in affected individuals.

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Area of Science:

Neurology
Ophthalmology
Cardiology

Background:

Kearns-Sayre syndrome is a rare multisystem disorder.
It is characterized by chronic progressive external ophthalmoplegia.

Observation:

Three patients (15-54 years) with Kearns-Sayre syndrome were studied.
All patients exhibited chronic progressive external ophthalmoplegia, retinal changes, and cardiac conduction defects.
Neurological signs included high-tone deafness and vestibular damage; elevated CSF protein was common.

Findings:

The syndrome presents with ophthalmoplegia, retinal degeneration, and cardiac arrhythmias.
Associated neurological, endocrine, and skeletal abnormalities can occur.
The etiology of Kearns-Sayre syndrome remains unknown, though neurogenic origins are suspected for ophthalmoplegia.

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Implications:

Prompt pacemaker implantation can mitigate risks associated with cardiac conduction defects, such as Adams-Stokes attacks.
Understanding the neurogenic basis of ophthalmoplegia is crucial for management.
Further research into the unknown cause of Kearns-Sayre syndrome is warranted.