High incidence of pre-excitation syndrome in Japanese families with Leber's hereditary optic neuropathy

Clin Genet. 1996 Dec;50(6):535-7. doi: 10.1111/j.1399-0004.1996.tb02732.x.


Cardiac conduction abnormalities have been reported in families with Leber's hereditary optic neuropathy (LHON). The pre-excitation syndrome Wolff-Parkinson-White syndrome or Lown-Ganong-Levine syndrome, is reportedly common in Finns with LHON, being seen in 14 (9%) of the 163 individuals with mitochondrial DNA (mtDNA) mutations. While this syndrome is thought to be rare in other ethnic groups with LHON, the present study of 35 Japanese LHON families confirmed that it is also relatively common among Japanese families, being seen in 5 (8%) of the 63 individuals with mtDNA mutations. It remains to be determined whether the high incidence of the pre-excitation syndrome in Finnish and Japanese LHON families is due to a particular genetic composition of ethnic groups such as in Finland and in Japan, or only to a reporting bias.

Publication types

  • Comparative Study

MeSH terms

  • Comorbidity
  • DNA, Mitochondrial / genetics
  • Female
  • Finland / epidemiology
  • Humans
  • Incidence
  • Japan / epidemiology
  • Male
  • Optic Atrophies, Hereditary / ethnology*
  • Wolff-Parkinson-White Syndrome / ethnology*


  • DNA, Mitochondrial