References

r-n-j

Российский неврологический журнал

Russian neurological journal

2658-79472686-7192

МИА

10.30629/2658-7947-2019-24-6-4-13

r-n-j-42

Research Article

ОБЗОРЫ

REVIEWS

Прогрессирующая наружная офтальмоплегия

Progressive external ophthalmoplegia

https://orcid.org/0000-0002-7514-5736

Яковенко

Е. В.

Iakovenko

E. V.

Яковенко Елена Владимировна — аспирант нейрогенетического отделения/

Москва.

Moscow.

helenaabracham@gmail.com

https://orcid.org/0000-0001-8070-7644

Федотова

Е. Ю.

Fedotova

E. Yu.

Москва.

Fedotova Ekaterina Yu. — Head of the Department of Neurogenetics in Research Center of Neurology.

Moscow.

ekfedotova@gmail.com

https://orcid.org/0000-0002-2704-6282

Иллариошкин

С. Н.

Illarioshkin

S. N.

Москва.

Moscow.

snillario@gmail.com

Научный центр неврологииРоссияResearch Center of NeurologyRussian Federation

2019

28012020

246413

2020

Яковенко Е.В., Федотова Е.Ю., Иллариошкин С.Н.

Iakovenko E.V., Fedotova E.Y., Illarioshkin S.N.

Данная работа распространяется под лицензией Creative Commons Attribution 4.0.

This work is licensed under a Creative Commons Attribution 4.0 License.

https://www.r-n-j.com/jour/article/view/42

Прогрессирующая наружная офтальмоплегия (ПНО) характеризуется прогрессирующим билатеральным птозом и нарушением подвижности глазных яблок в горизонтальном и вертикальном направлениях и представляет собой характерный симптом многих митохондриальных заболеваний, а не отдельную нозологическую форму. ПНО часто возникает совместно с другими системными проявлениями митохондриальной дисфункции. Правильная и ранняя диагностика ПНО первостепенна для оптимального ведения данных пациентов. В статье представлен обзор последних данных по этиологии, спектру клинических проявлений, дифференциальному диагнозу, методам диагностики и лечебным опциям для изолированной хронической ПНО и других ПНО-ассоциированных митохондриальных синдромов.

Progressive external ophthalmoplegia (PEO) is characterized by progressive bilateral ptosis and eye movement disturbances in horizontal and vertical directions and represents a typical symptom of many mitochondrial disorders, but not a separate disease. PEO often occurs together with other systemic manifestations of mitochondrial disfunction. Correct and early diagnosis of PEO is essential for optimal management of these patients. In this review we collected the last data about etiology, spectrum of clinical symptoms, differential diagnosis, diagnostical methods and treatment options for isolated chronic PEO and other PEO-associated mitochondrial syndromes.

прогрессирующая наружная офтальмоплегиямитохондриальное заболеваниесиндром Кернса–Сейраофтальмоплегиямиопатия

progressive external ophthalmoplegiamitochondrial disorderKearns–Sayre syndromeophthalmoplegiamyopathy

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The authors declare that there are no conflicts of interest present.