Late onset Leigh syndrome: about an observation

Leigh Syndrome (LS) is a rare childhood mitochondriopathy characterized by clinical polymorphism. Generally, it begins at the age of 2 years .We report the case of a 13 year old patient, born from on inbred marriage,she resented a significant decrease of visual acuity at the age of 9 years. Toward...

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Main Authors: ahira BARKA-BEDRANE, Djaoued BOUCHENAK-KHELLADI, Cherifa LOUHIBI, Hakim CHIALI
Format: Article
Language:English
Published: University of Oran 1 2020-12-01
Series:Journal de la Faculté de Médecine d'Oran
Subjects:
Online Access:https://www.ajol.info/index.php/jfmo/article/view/226768
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author ahira BARKA-BEDRANE
Djaoued BOUCHENAK-KHELLADI
Cherifa LOUHIBI
Hakim CHIALI
author_facet ahira BARKA-BEDRANE
Djaoued BOUCHENAK-KHELLADI
Cherifa LOUHIBI
Hakim CHIALI
author_sort ahira BARKA-BEDRANE
collection DOAJ
description Leigh Syndrome (LS) is a rare childhood mitochondriopathy characterized by clinical polymorphism. Generally, it begins at the age of 2 years .We report the case of a 13 year old patient, born from on inbred marriage,she resented a significant decrease of visual acuity at the age of 9 years. Towards the age of 13 years, she presented bulbar and walking disorders. The neurological examination found a ppyramidal, an extrapyramidal, a neurogenic syndromes and a kyphoscoliosis. Brain MRI objectified a bilateral and symmetrical hyper signal in the brainstem, the basal ganglia and the periaqueductal region. Brain spectro-MRI found a peak in lactates.
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spelling doaj.art-a64a3a96fe494df9bf56ecf68d7b56ab2023-08-18T19:41:54ZengUniversity of Oran 1Journal de la Faculté de Médecine d'Oran2571-98742602-65112020-12-0142Late onset Leigh syndrome: about an observation ahira BARKA-BEDRANE0Djaoued BOUCHENAK-KHELLADI1Cherifa LOUHIBI2Hakim CHIALI3Service de neurologie Centre hospitalo universitaire Dr Tidjani Damerdji Tlemcen Faculté de médecine, université Abou Bekr Belkaid TlemcenService de neurologie Centre hospitalo universitaire Dr Tidjani Damerdji Tlemcen Faculté de médecine, université Abou Bekr Belkaid TlemcenService de neurologie Centre hospitalo universitaire Dr Tidjani Damerdji TlemcenFaculté de médecine, université Abou Bekr Belkaid TlemcenService de neurologie Centre hospitalo universitaire Dr Tidjani Damerdji Tlemcen Faculté de médecine, université Abou Bekr Belkaid Tlemcen Leigh Syndrome (LS) is a rare childhood mitochondriopathy characterized by clinical polymorphism. Generally, it begins at the age of 2 years .We report the case of a 13 year old patient, born from on inbred marriage,she resented a significant decrease of visual acuity at the age of 9 years. Towards the age of 13 years, she presented bulbar and walking disorders. The neurological examination found a ppyramidal, an extrapyramidal, a neurogenic syndromes and a kyphoscoliosis. Brain MRI objectified a bilateral and symmetrical hyper signal in the brainstem, the basal ganglia and the periaqueductal region. Brain spectro-MRI found a peak in lactates. https://www.ajol.info/index.php/jfmo/article/view/226768Leigh syndromelate onsetrare mitochondrial disorder
spellingShingle ahira BARKA-BEDRANE
Djaoued BOUCHENAK-KHELLADI
Cherifa LOUHIBI
Hakim CHIALI
Late onset Leigh syndrome: about an observation
Journal de la Faculté de Médecine d'Oran
Leigh syndrome
late onset
rare mitochondrial disorder
title Late onset Leigh syndrome: about an observation
title_full Late onset Leigh syndrome: about an observation
title_fullStr Late onset Leigh syndrome: about an observation
title_full_unstemmed Late onset Leigh syndrome: about an observation
title_short Late onset Leigh syndrome: about an observation
title_sort late onset leigh syndrome about an observation
topic Leigh syndrome
late onset
rare mitochondrial disorder
url https://www.ajol.info/index.php/jfmo/article/view/226768
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AT djaouedbouchenakkhelladi lateonsetleighsyndromeaboutanobservation
AT cherifalouhibi lateonsetleighsyndromeaboutanobservation
AT hakimchiali lateonsetleighsyndromeaboutanobservation