2013
DOI: 10.1001/2013.jamaneurol.139
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Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

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Cited by 23 publications
(9 citation statements)
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“…In sCJD of the MV2 subtype, as was our patient, kuru plaques in the cerebellum are also frequent [ 4 ], but the pattern of plaques in our case was clearly different. Our patient also had a prolonged course of almost five years, which is atypical for CJD of sporadic and iatrogenic cause, but there is a case report of a 56-year-old man who had a 42-month course of CJD, also with an MV2 subtype and presenting as primary progressive aphasia [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
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“…In sCJD of the MV2 subtype, as was our patient, kuru plaques in the cerebellum are also frequent [ 4 ], but the pattern of plaques in our case was clearly different. Our patient also had a prolonged course of almost five years, which is atypical for CJD of sporadic and iatrogenic cause, but there is a case report of a 56-year-old man who had a 42-month course of CJD, also with an MV2 subtype and presenting as primary progressive aphasia [ 12 ].…”
Section: Discussionmentioning
confidence: 99%
“…Her pathology did not have a “classic” iCJD phenotype, but it was also not typical for sCJD, given the multicentric plaques in the cerebellum. The long symptomatic period may have allowed more severe pathology to develop [ 13 ], but the plaque pattern seen in our patient was not described in the long duration MV2 case with primary progressive aphasia [ 12 ]. Finally, whether the inflammation from PACNS predisposed that area of the brain to prion propagation is another interesting speculation.…”
Section: Discussionmentioning
confidence: 99%
“…The only alternative diagnosis to consider is Creutzfeldt–Jakob disease, which can cause a focal progressive aphasia that may evolve over a period of 3 or 4 years but almost never over a period of 8 years. 1,2 …”
Section: Clinical Differential Diagnosismentioning
confidence: 99%
“…Mesulam Ce patient porte é galement une mutation de la progranuline, ce qui pose la question d'une potentielle interaction des diffé rentes proté ines à l'origine des lé sions observé es chez lui [97]. Enfin, 3 cas de neuropathologie de maladie de CreutzfeldtJakob sont rapporté es chez des patients ayant pré senté un syndrome logopé nique typique é voluant de maniè re isolé e durant une longue pé riode, respectivement 18 mois, 3,5 ans et 2 ans [99,102,103]. …”
Section: Particularite´s Neuropathologiquesunclassified