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Infantile Type Sandhoff Disease with Striking Brain MRI Findings and a Novel Mutation

BACKGROUND: Sandhoff disease is an autosomal recessive disorder caused by β-hexosaminidase deficiency in which the ganglioside GM2 and other glycolipids accumulate intracellularly within lysosomes. This process results in progressive motor neuron manifestations, death from respiratory failure and in...

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Foilsithe in:Pol J Radiol
Main Authors: Beker-Acay, Mehtap, Elmas, Muhsin, Koken, Resit, Unlu, Ebru, Bukulmez, Aysegul
Formáid: Artigo
Teanga:Inglês
Foilsithe: International Scientific Literature, Inc. 2016
Ábhair:
Rochtain Ar Líne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4780271/
https://ncbi.nlm.nih.gov/pubmed/26985245
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.12659/PJR.895911
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