Conditions / Musculoskeletal

Kearns-Sayre syndrome

info · Musculoskeletal · ICD-10: H49.81

A chronic progressive external ophthalmoplegia characterized by progressive external ophthalmoplegia, pigmentary retinopathy, and one or more of heart block, cerebellar ataxia, or a cerebrospinal fluid protein level above 100 mg/dl, that has_material_basis_in

A chronic progressive external ophthalmoplegia characterized by progressive external ophthalmoplegia, pigmentary retinopathy, and one or more of heart block, cerebellar ataxia, or a cerebrospinal fluid protein level above 100 mg/dl, that has_material_basis_in deletion of mitochondrial DNA. Onset is before 20 years of age.

Signs and symptoms

  • Short stature
  • Seizure
  • Pigmentary retinopathy
  • Ataxia
  • Arrhythmia
  • Diabetes mellitus
  • Third degree atrioventricular block
  • Renal tubular acidosis
  • Sideroblastic anemia
  • Muscle weakness

Also known as: KSS