Kearns-Sayre syndrome (KSS)
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Kearns-Sayre syndrome (KSS). Progressive external ophthalmoplegia (PEO), Pigmentary retinopathy. Fully referenced neurology checklist.
Diagnostic triad
- Progressive external ophthalmoplegia (PEO)
- Pigmentary retinopathy
- Cardiac conduction defects
Ophthalmic features
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Neurological features
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Cardiac features
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Endocrine features
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Systemic features
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Differential diagnosis
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Magnetic resonance imaging (MRI): feature
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Other investigations
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Treatments
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References
- Rahman S, Hanna MG. Diagnosis and therapy in neuromuscular disorders: diagnosis and new treatments in mitochondrial diseases. JNNP 2009; 80:943-953.
- Tsang SH, Aycinena ARP, Sharma T. Mitochondrial disorder: Kearns-Sayre syndrome. Adv Exp Med Biol 2018; 1085:161-162.
- Sharma AK, Jain N, Kharwar RB, Narain VS. Classical triad of Kearns-Sayre syndrome. BMJ Case Rep 2016; 2016:bcr2016216500.
- Nguyen MTB, Micieli J, Margolin E. Teaching NeuroImages: Kearns-Sayre syndrome. Neurology 2019; 92:e519-e520.
- Diamantopoulou P, Ward VM, Harries ML. Kearns-Sayre syndrome: presenting with vocal fold palsy. J Laryngol Otol 2001; 115:1021-1022.
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