نتایج جستجو برای: neurometabolic disorders

تعداد نتایج: 671446  

2016
Taraka R. Donti Gerarda Cappuccio Leroy Hubert Juanita Neira Paldeep S. Atwal Marcus J. Miller Aaron L. Cardon V. Reid Sutton Brenda E. Porter Fiona M. Baumer Michael F. Wangler Qin Sun Lisa T. Emrick Sarah H. Elsea

Adenylosuccinate lyase (ADSL) deficiency is a rare autosomal recessive neurometabolic disorder that presents with a broad-spectrum of neurological and physiological symptoms. The ADSL gene produces an enzyme with binary molecular roles in de novo purine synthesis and purine nucleotide recycling. The biochemical phenotype of ADSL deficiency, accumulation of SAICAr and succinyladenosine (S-Ado) i...

2014
Parveneh KARIMZADEH Farzad AHMADABADI Narjes JAFARI Fakhreddin SHARIATMADARI Hamid NEMATI Adel AHADI Sanaz KARIMI DARDASHTI Mehrdad MIRZARAHIMI Zahra DASTBORHAN Javad ZARE NOGHABI

OBJECTIVE Phenylketonuria is one of the most common metabolic disorders and the first known cause of mental retardation in pediatrics. As Screening for phenylketonuria (PKU) is not a routine neurometabolic screening test for neonates in Iran, many PKU cases may be diagnosed after developing the clinical symptoms. One of the findings of PKU is myelination disorders, which is seen as hypersignal ...

Journal: :Neurology, Neuropsychiatry, Psychosomatics 2019

2016
Dominique Endres Ludger Tebartz van Elst Bernd Feige Stephan Backenecker Kathrin Nickel Anna Bubl Thomas Lange Irina Mader Simon Maier Evgeniy Perlov

In neuropsychiatric research, the aspects of sex have received increasing attention over the past decade. With regard to the neurometabolic differences in the prefrontal cortex and the cerebellum of both men and women, we performed a magnetic resonance spectroscopic (MRS) study of a large group of healthy subjects. For neurometabolic measurements, we used single-voxel proton MRS. The voxels of ...

2014
Parvaneh KARIMZADEH Narjes JAFARI Farzad AHMAD ABADI Sayena JABBEDARI Mohammad-Mahdi TAGHDIRI Mohammad-Reza ALAEE Mohammad GHOFRANI Seyed Hassan TONEKABONI Habibeh NEJAD BIGLARI

OBJECTIVE Propionic acidemia is one of the rare congenital neurometabolic disorders with autosomal recessive inheritance. This disorder is caused by a defect in the propionyl-CoA carboxylase enzyme and can be presented with life-threatening ketoacidosis, lethargy, failure to thrive, and developmental delay. MATERIALS & METHODS The patients diagnosed as having propionic acidemia in Neurology D...

Journal: :iranian journal of child neurology 0
farzad ahmadabadi pediatric neurology department, ardabil university of medical sciences, ardabil, iran

how to cite this article: ahmadabadi f. neurometabolic registry site in iran (leukodystrophies). iran j child neurol. autumn 2014;8;4(suppl.1):22. pls see pdf.

Journal: :Frontiers in Pharmacology 2021

The neurobiological bases of mood instability are poorly understood. Neuronal network alterations and neurometabolic abnormalities have been implicated in the pathophysiology anxiety conditions associated with hence candidate mechanisms underlying its neurobiology. Fast-spiking parvalbumin GABAergic interneurons modulate activity principal excitatory neurons through their inhibitory action dete...

Journal: :Current Tissue Microenvironment Reports 2020

2016
Jean-Pierre Lin Nardo Nardocci

Dystonia in childhood may be severely disabling and often unremitting and unrecognized. Considered a rare disorder, dystonic symptoms in childhood are pervasive in many conditions including disorders of developmental delay, cerebral palsy (CP), autism, neurometabolic, neuroinflammatory, and neurogenetic disorders. Collectively, there is a need to recognize the role of early postures and movemen...

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