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10.1016/j.neurobiolaging.2014.04.009 http://scihub22266oqcxt.onion/10.1016/j.neurobiolaging.2014.04.009 C4087047!4087047!24819148     free     free     free Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Deprecated: Implicit conversion from float 233.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534       Neurobiol+Aging 2014 ; 35 (10): 2419.e17-21 Nephropedia Template TP {{tp|p=24819148|t=2014. Hippocampal sclerosis dementia with the C9ORF72 hexancleotide repeat expansion |pdf=|usr=}}{{24819148}} gab.com Text Hippocampal sclerosis dementia with the C9ORF72 hexancleotide repeat expansion JO: Neurobiol Aging http://www.kidney.de/pget.php?&tw=1&pmid=24819148 #FOAvip Frontotemporal dementia and amyotrophic lateral sclerosis are the main syndromes of the chromosome 9 ORF72 (C9ORF72) hexanucleotide repeat expansion, but studies have shown a substantial phenotypic diversity that includes psychiatric presentations. This study describes hippocampal sclerosis dementia (HSD) in carriers of the C9ORF72 mutation. We compared clinical and neuropathological features of HSD in carriers and non-carries autopsied at Johns Hopkins. Carriers presented with amnesia, agitation, dissocial behavior and impaired self-care, whereas non-carriers showed little agitation. The groups were not dissimilar in cognitive or motor dysfunction. Neuropathological examination of carriers showed cerebellar neuronal inclusions positive for ubiquitin, p62, and ubiquilin-2, and negative for TAR DNA-binding protein 43. Non-carriers did not have cerebellar inclusions. C9ORF72 repeat-associated non-ATG (RAN) translation was confirmed by immunohistochemistry.These observations broaden the C9ORF72 phenotype and place HSD in the FTD spectrum. The amnesic phenotype of HSD, which is consistent with the focal hippocampal atrophy, should be included in clinical categorizations of FTD. Twit Text FOAVip Hippocampal sclerosis dementia with the C9ORF72 hexancleotide repeat expansion ????Neurobiol Aging http://www.kidney.de/pget.php?&tw=1&pmid=24819148 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=24819148 #FOAvip English Wikipedia <ref>{{Cite pmid|24819148}}</ref> Hippocampal sclerosis dementia with the C9ORF72 hexancleotide repeat expansion #MMPMID24819148 Pletnikova O; Sloane KL; Renton AE; Traynor BJ; Crain BJ; Reid T; Zu T; Ranum LPW; Troncoso JC; Rabins PV; Onyike CU Neurobiol Aging 2014[Oct]; 35 (10): 2419.e17-21 PMID24819148show ga Frontotemporal dementia and amyotrophic lateral sclerosis are the main syndromes of the chromosome 9 ORF72 (C9ORF72) hexanucleotide repeat expansion, but studies have shown a substantial phenotypic diversity that includes psychiatric presentations. This study describes hippocampal sclerosis dementia (HSD) in carriers of the C9ORF72 mutation. We compared clinical and neuropathological features of HSD in carriers and non-carries autopsied at Johns Hopkins. Carriers presented with amnesia, agitation, dissocial behavior and impaired self-care, whereas non-carriers showed little agitation. The groups were not dissimilar in cognitive or motor dysfunction. Neuropathological examination of carriers showed cerebellar neuronal inclusions positive for ubiquitin, p62, and ubiquilin-2, and negative for TAR DNA-binding protein 43. Non-carriers did not have cerebellar inclusions. C9ORF72 repeat-associated non-ATG (RAN) translation was confirmed by immunohistochemistry.These observations broaden the C9ORF72 phenotype and place HSD in the FTD spectrum. The amnesic phenotype of HSD, which is consistent with the focal hippocampal atrophy, should be included in clinical categorizations of FTD. äHippocampal sclerosis dementia with the C9ORF72 hexancleotide repeat e(epmc).pdf DeepDyve Pubget Overpricing