نتایج جستجو برای: leigh syndrome

تعداد نتایج: 623686  

Journal: :Annals of Indian Academy of Neurology 2007

2017
Joyeeta Rahman Alberto Noronha Ines Thiele Shamima Rahman

Mitochondrial disorders are among the most severe metabolic disorders wherein patients suffer from multisystemic phenotypes, often resulting in early death. Clinical, biochemical, and genetic heterogeneity among individuals, together with poor understanding of gene-tophenotype relationships, pose significant diagnostic and therapeutic challenges for clinicians. In light of recent advances in ne...

2016
Miguel Chuquilin Raghav Govindarajan Dawn Peck Esperanza Font-Montgomery

Leigh syndrome is a mitochondrial disease caused by mutations in different genes, including ATP6A for which no known therapy is available. We report a case of adult-onset Leigh syndrome with response to immunotherapy. A twenty year-old woman with baseline learning difficulties was admitted with progressive behavioral changes, diplopia, headaches, bladder incontinence, and incoordination. Brain ...

Journal: :Russkij žurnal detskoj nevrologii 2023

Mitochondrial dysfunction mainly affects organs with high metabolic demand, primarily the brain. Epilepsy is a common phenotypic sign of both syndromic and non-syndromic mitochondrial diseases. in diseases poorly treatable often an unfavorable prognostic sign. Patients epilepsy are prescribed various anti-seizure medications. Mitochondriotoxic drugs should be avoided. Descriptions MELAS-syndrom...

Journal: :The Pan African medical journal 2015
Leila Mnif Rim Sellami Jawaher Masmoudi

Leigh syndrome is a mitochondrial encephalomyopathy that occurs due to "cytochrome c oxidase deficiency". Few psychiatric disorders have been defined that are associated with Leigh syndrome. The objective of this work is to study relations between mitochondrial dysfunction and psychiatric disorders. It was a 20 year old male patient, who received Modopar, for severe extra pyramidal symptoms cau...

Journal: :JPMA. The Journal of the Pakistan Medical Association 2013
Nesat Cullu Ekrem Karakas Omer Karakas Mehmet Deveer Mustafa Calik Fatima Nurefsan Boyaci

Moyamoya disease is a cerebrovasculopathy of unknown etiology during the course of which the main and terminal veins of the internal carotid artery undergo progressive vein occlusion. Leigh syndrome is a mitochondrial encephalomyopathy that occurs due to "cytochrome c oxidase deficiency" characterized by psychomotor retardation, difficulty in eating, seizures, hypotonia, respiratory disorders a...

Journal: :Journal of medical genetics 2003
M Chol S Lebon P Bénit D Chretien P de Lonlay A Goldenberg S Odent L Hertz-Pannier C Vincent-Delorme V Cormier-Daire P Rustin A Rötig A Munnich

Leigh syndrome is a subacute necrotising encephalomyopathy frequently ascribed to mitochondrial respiratory chain deficiency. This condition is genetically heterogeneous, as mutations in both mitochondrial (mt) and nuclear genes have been reported. Here, we report the G13513A transition in the ND5 mtDNA gene in three unrelated children with complex I deficiency and a peculiar MRI aspect distinc...

2010
Dhananjay Y Shrikhande Piyush Kalakoti MM Aarif Syed Kunal Ahya Gurmeet Singh

Leigh syndrome is a rare progressive neurodegenerative, mitochondrial disorder of childhood with only a few cases documented from India. The clinical presentation of Leigh syndrome is highly variable. However, in most cases it presents as a progressive neurological disease with motor and intellectual developmental delay and signs and symptoms of brain stem and/or basal ganglia involvement. Rais...

Journal: :Pediatric Neurology Briefs 1987

Journal: :Journal of Biological Chemistry 2014

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید