نتایج جستجو برای: neurometabolic disorder

تعداد نتایج: 597203  

Journal: :iranian journal of child neurology 0
narjes jafari pediatric neurology research center, shahid beheshti university of medical sciences, tehran, iran

how to cite this article: jafari n. neurometabolic disorders, clinical and neuroimaging finding. iran j child neurol. autumn 2014;8;4(suppl.1):23. pls see pdf.

2016
Emma S. Reid Apostolos Papandreou Suzanne Drury Christopher Boustred Wyatt W. Yue Yehani Wedatilake Clare Beesley Thomas S. Jacques Glenn Anderson Lara Abulhoul Alex Broomfield Maureen Cleary Stephanie Grunewald Sophia M. Varadkar Nick Lench Shamima Rahman Paul Gissen Peter T. Clayton Philippa B. Mills

Neurometabolic disorders are markedly heterogeneous, both clinically and genetically, and are characterized by variable neurological dysfunction accompanied by suggestive neuroimaging or biochemical abnormalities. Despite early specialist input, delays in diagnosis and appropriate treatment initiation are common. Next-generation sequencing approaches still have limitations but are already enabl...

2014
Parvaneh KARIMZADEH Narjes JAFARI Habibeh NEJAD BIGLARI Sayena JABBEH DARI Farzad AHMAD ABADI Mohammad-Reza ALAEE Hamid NEMATI Sasan SAKET Seyed Hasan TONEKABONI Mohammad-Mahdi TAGHDIRI Mohammad GHOFRANI

OBJECTIVE GM2-Gangliosidosis disease is a rare autosomal recessive genetic disorder that includes two disorders (Tay-Sachs and Sandhoff disease).These disorders cause a progressive deterioration of nerve cells and inherited deficiency in creating hexosaminidases A, B, and AB. MATERIALS & METHODS Patients who were diagnosed withGM2-Gangliosidosis in the Neurology Department of Mofid Children's...

2013
Parvaneh KARIMZADEH Farzad AHMADABADI Narjes JAFARI Sayena JABBEHDARI Mohammad Reza ALAEE Mohammad GHOFRANI Mohammad Mahdi TAGHDIRI Seyed Hassan TONEKABONI

OBJECTIVE Biotinidase deficiency is one of the rare congenital metabolic disorders with autosomal recessive inheritance. If this disorder is diagnosed in newborn period, could be prevented well from mental and physical developmental delay and most of clinical manifestations. MATERIALS & METHODS The patients were diagnosed as biotinidase deficiency in Neurology Department of Mofid Children's H...

2011
Luke C Henry Sébastien Tremblay Suzanne Leclerc Abdesselam Khiat Yvan Boulanger Dave Ellemberg Maryse Lassonde

BACKGROUND Despite negative neuroimaging findings many athletes display neurophysiological alterations and post-concussion symptoms that may be attributable to neurometabolic alterations. METHODS The present study investigated the effects of sports concussion on brain metabolism using 1H-MR Spectroscopy by comparing a group of 10 non-concussed athletes with a group of 10 concussed athletes of...

Journal: :The American journal of clinical nutrition 2009
Pierre J Magistretti

The coupling between synaptic activity and glucose utilization (neurometabolic coupling) is a central physiologic principle of brain function that has provided the basis for 2-deoxyglucose-based functional imaging with positron emission tomography. Approximately 10 y ago we provided experimental evidence that indicated a central role of glutamate signaling on astrocytes in neurometabolic coupli...

2016
Julián Benito-León Elan D. Louis Virginia Mato-Abad Ulrike Dydak Juan Álvarez-Linera Juan Antonio Hernández-Tamames José Antonio Molina-Arjona Norberto Malpica Michele Matarazzo Juan Pablo Romero Álvaro Sánchez-Ferro

The pathogenesis of orthostatic tremor (OT) remains unclear, although some evidence points to dysfunction in the brainstem or cerebellum. We used single voxel proton magnetic resonance spectroscopy (1H-MRS) (3 T) to investigate whether neurochemical changes underlie abnormal cerebellar or cortical function in OT. Fourteen OT patients and 14 healthy controls underwent 1H-MRS studies with voxels ...

نمودار تعداد نتایج جستجو در هر سال

با کلیک روی نمودار نتایج را به سال انتشار فیلتر کنید