نتایج جستجو برای: mitochondrial encephalomyopathy

تعداد نتایج: 132426  

Journal: :acta medica iranica 0
p. ayatollahi a. tarazi s. nafissi

mitochondrial neurogastrointestinal encephalo-myopathy (mngie) is a rare autosomal recessive disease caused by thymidine phosphorylase (tp) gene mutation. here we report a patient with mngie in whom sensorimotor polyneuropathy was the first presenting symptom and had a fluctuating course. this 26-year-old female patient developed acute-onset demyelinating polyneuropathy from the age of 6 with t...

Journal: :iranian journal of child neurology 0
mahmoud reza ashrafi 1. pediatric neurology department, children’s medical center, pediatric center of excellence, tehran university of medical science, tehran, iran alireza tavasoli 1. pediatric neurology department, children’s medical center, pediatric center of excellence, tehran university of medical science, tehran, iran

pls see pdf.

Journal: :AJNR. American journal of neuroradiology 1991
F S Sandhu W P Dillon

The mitochondrial encephalomyopathies are a heterogeneous group of disorders characterized by mitochondrial dysfunction that produce multisystem symptoms. This disorder affects both the central and peripheral nervous systems, skeletal muscles, heart, endocrine glands, gastrointestinal tract, hematopoietic system, and kidneys (Table 1) [1]. Patients with mitochondrial encephalomyopathy can be di...

Journal: :The journal of international advanced otology 2018
Teruo Toi Yasuyuki Nomura Akihiro Kishino Shuntaro Shigihara Takeshi Oshima Harumi Ishikawa Satoshi Kamei Hidemi Miyazaki

Cases of dizziness caused by multiple sclerosis are commonly reported, but those caused by mitochondrial encephalomyopathy have been rarely reported. Particularly, the description of eye nystagmography (ENG) using caloric and optokinetic nystagmus tests has not been reported to date. We encountered the case of a 40-year-old woman with mitochondrial encephalomyopathy who visited us with the chie...

We read with interest the article by Almasi et al. on a 48 years old female patient with Mitochondrial Encephalomyopathy, Lactic Acidosis, And Stroke-like episodes (MELAS), diagnosed based on the clinical presentation, blood test results, and imaging and muscle biopsy findings . We have the following comments and concerns.

Journal: :JIMD reports 2017
Sarah U Morton Edward G Neilan Roy W A Peake Jiahai Shi Klaus Schmitz-Abe Meghan Towne Kyriacos Markianos Sanjay P Prabhu Pankaj B Agrawal

Early-onset mitochondrial encephalomyopathy is a rare disorder that presents in the neonatal period with lactic acidosis, hypotonia, and developmental delay. Sequence variants in the nuclear-encoded gene FBXL4 have been previously demonstrated to be a cause of early-onset mitochondrial encephalomyopathy in several unrelated families. We have identified a pair of siblings with mutations in FBXL4...

Journal: :Brain : a journal of neurology 2015
Joerg P Halter W Michael M Schüpbach Hanna Mandel Carlo Casali Kim Orchard Matthew Collin David Valcarcel Attilio Rovelli Massimiliano Filosto Maria T Dotti Giuseppe Marotta Guillem Pintos Pere Barba Anna Accarino Christelle Ferra Isabel Illa Yves Beguin Jaap A Bakker Jaap J Boelens Irenaeus F M de Coo Keith Fay Carolyn M Sue David Nachbaur Heinz Zoller Claudia Sobreira Belinda Pinto Simoes Simon R Hammans David Savage Ramon Martí Patrick F Chinnery Ronit Elhasid Alois Gratwohl Michio Hirano

Haematopoietic stem cell transplantation has been proposed as treatment for mitochondrial neurogastrointestinal encephalomyopathy, a rare fatal autosomal recessive disease due to TYMP mutations that result in thymidine phosphorylase deficiency. We conducted a retrospective analysis of all known patients suffering from mitochondrial neurogastrointestinal encephalomyopathy who underwent allogenei...

Journal: :Journal of Neurology, Neurosurgery & Psychiatry 1989

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