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Kearns-sayre Syndrome Treated with Permanent Pacemaker Insertion for Complete Atrioventricular Block

Park EH, Her SH, Ha MA, Kim HS, Jang JH

Kearns-Sayre syndrome (KSS) is a rare multisystem mitochondrial disorder associated with progressive external ophthalmoplegia, atypical pigmentary degeneration of the retina, and complete heart block. KSS can lead to a risk...
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Clinical manifestations and anesthetic management of Kearns-Sayre syndrome: A case report

Lee HM, Heo SJ, Jee DL

  • KMID: 1741923
  • Anesth Pain Med.
  • 2011 Jul;6(3):290-293.
Kearns-Sayre syndrome (KSS) is a mitochondrial disorder resulting in multi-system dysfunction. A 14-year-old boy with KSS underwent external levator muscle resection for correction of ptosis. There were no abnormalities on...
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Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report

Faris AS, Tawfic QA, Jeyaraj L

Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy,...
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Kearns-Sayre Syndrome with a Large Deletion in Mitochondrial DNA

Kim SH, Hwang JH, Chung KW, Kim HJ, Kim JY, Park KD, Sunwoo IN, Choi BO

  • KMID: 1572460
  • J Korean Neurol Assoc.
  • 2006 Jun;24(3):260-264.
Mitochondrial DNA (mtDNA) deletions have been found in a majority of patients with Kearns-Sayre syndrome (KSS). The proband, a 14-year-old male, presented with retinitis pigmentosa, bilateral ptosis with an external...
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Treatment of Eyelid Ptosis due to Kearns-Sayre Syndrome Using Frontalis Suspension

Weitgasser L, Wechselberger G, Ensat F, Kaplan R, Hladik M

Blepharoptosis is a common indication for surgery in plastic surgery units, yet its possible underlying pathology frequently remains unidentified. A 52-year-old man with a 20-year history of progressive bilateral ptosis...
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MRI follow-up study and 1H-MR spectroscopic finding in a patient with Kearns-Sayre syndrome

Choi C, Sunwoo IN, Kim TS, Jeon P

  • KMID: 2342676
  • J Korean Neurol Assoc.
  • 1998 Jun;16(3):388-391.
Serial Brain MRI was performed on a seventeen-year-old girl with Kearns-Sayre syndrome. At the age of 11, she complained bilateral ptosis. Two years later, bilateral blepharoplasty was done and brain...
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Clinical Phenotypic Patterns of Ophthalmoplegia Plus with Ragged Red Fiber

Lee KW, Kim SY, Kim JY, Lee NS, Kim SH, Lee SB, Myung HJ, Chi JG

  • KMID: 2016121
  • J Korean Neurol Assoc.
  • 1992 Jun;10(2):224-231.
Chronic progressive ophthalmoplegia(CPEO) is a vague clinical entity, which needs further understanding and there is still intense controversy about the classification on the Syndrome of Progressive External Ophthalmoplegia. In our...
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A Case of the Hypercalcemia Induced by the Coenzyme Q10 and Alphacalcidol Treatment in a Patient with Kearns-Sayre Syndrome and Hypoparathyroidism

Jung HH, Hwang HH, Kim DH

  • KMID: 1520747
  • J Korean Soc Pediatr Endocrinol.
  • 2007 Dec;12(2):155-158.
Kearns-Sayre Syndrome (KSS) is rare mitochondrial disorder characterized by chronic progressive external ophthalmoplegia, atypical retinal pigmentation and complete heart block. It is occasionally combined endocrinologic symptoms such as hypoparathyroidism, short...
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Vascular Hyperemia and Crossed Cerebellar Diaschisis in MELAS Patient Presented as Stroke-Like Episode and Seizure

Kim DW, Choi KH, Oh HJ, Kang M, Kim C, Choi HC, Sohn JH

  • KMID: 2184752
  • J Korean Neurol Assoc.
  • 2013 Aug;31(3):183-185.
Mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) is one of the mitochondrial disorders that can present as a stroke-like episode or seizure. Although the pathophysiology of MELAS remains inconclusive,...
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A case of KearnsSayre syndrome complicated with complete AV block

Park IH, Lim JK, Moon JG, Joung BY, Lee MH, Kim SS

  • KMID: 2305997
  • Korean J Med.
  • 2006 May;70(5):564-568.
KearnsSayre syndrome (KSS) is a mitochondrial DNA disorder characterized by the onset before age 20 years, progressive external opthalomoplegia, atypical retinal pigmentation and cardiac conduction disturbance. This report describes a...
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Autosomal Dominant Type of Chronic Progressive External Ophthalmoplegia With Elevated Acetylcholine Receptor Binding Antibody

Ko MS, Kim MS, Kwon JH, Kim WJ, Lee EM, Kim SY, Kim DS

  • KMID: 1966528
  • J Korean Neurol Assoc.
  • 2012 Aug;30(3):214-217.
External ophthalmoplegia and ptosis are common manifestations of mitochondrial cytopathy, such as chronic progressive external ophthalmoplegia (CPEO). However, these symptoms and signs may also be presenting features of myasthenia gravis...
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Ophthalmoplegia in Mitochondrial Disease

Lee SJ, Na JH, Han J, Lee YM

PURPOSE: To evaluate the classification, diagnosis, and natural course of ophthalmoplegia associated with mitochondrial disease. MATERIALS AND METHODS: Among 372 patients with mitochondrial disease who visited our hospital between January 2006...
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Large Scale Single Deletion of Mitochondrial DNA in Chronic Progressive External Ophthalmoplegia

Koh KN, Park SY, Hwang H, Chae JH, Choi JE, Kim KJ, Hwang YS

  • KMID: 1582211
  • J Korean Child Neurol Soc.
  • 2004 May;12(1):43-49.
PURPOSE: Chronic progressive external ophtahlmoplegia(CPEO) is a common phenotype of mitochondrial myopathy. CPEO has wide clinical spectrum with variable severity and can be divided into 3 groups; Kearns-Sayre syndrome, ophthalmoplegia...
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Clinical and Laboratorial Characteristics of Korean Children with Mitochondrial Respiratory Chain Defect

Noh BH, Lee YM, Seo JH, Hur YJ, Jung DE, Lee JS, Kim HD

  • KMID: 2329572
  • J Korean Child Neurol Soc.
  • 2006 Nov;14(2):207-214.
PURPOSE:The study was carried out to characterized the clinical and the laboratorial features of children with mitochondrial respiratoy chain disorders in Korea. METHODS:We retrospectively analyzed the clinical and the loboratorial data...
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Clinical and Laboratory Features of Children with Mitochondrial Respiratory Chain Enzyme Complexes Defect and Neurological Abnormalities: A case report

Ahn SJ, Park ES, Lee YM, Kim SH, Kim DJ, Rha DW

  • KMID: 2394071
  • J Korean Acad Rehabil Med.
  • 2009 Feb;33(1):118-122.
Mitochondrial cytopathies represent a heterogeneous group of multisystem disorder that preferentially affects the muscle and nervous systems. Mitochondrial respiratory chain enzyme complexes (MRC) defect can be the cause of many...
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