Creutzfeldt-Jakob Disease Revealed by a Logopenic Variant of Primary Progressive AphasiaMartory M.D.a · Roth S.d · Lövblad K.O.b · Neumann M.e · Lobrinus J.A.c · Assal F.a
Departments of aNeurology, bNeuroradiology and cPathology, Geneva University Hospitals, dClinique de Carouge, Geneva, and eDepartment of Pathology, Zurich University Hospital, Zurich, Switzerland
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Logopenic aphasia, mainly characterized by word anomia, sentence and phrase comprehension difficulties secondary to phonological loop deficits but relatively preserved single word comprehension and no agrammatism, is one of the 3 main variants of primary progressive aphasia (PPA). We describe the first case of PPA that fulfilled clinical criteria of logopenic aphasia but showed abnormal DWI hyperintensities that were predominant on the left hemisphere and compatible with Creutzfeldt-Jakob disease (CJD). After abnormally long isolated language deficits, the patient rapidly worsened and died. Autopsy performed 18 months after onset of language difficulties confirmed the diagnosis. We therefore advocate performing DWI sequences in any suspicion of PPA in order to rule out CJD.
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- Mesulam MM: Slowly progressive aphasia without generalized dementia. Ann Neurol 1982;11:592–598.
- Mesulam MM: Primary progressive aphasia. Ann Neurol 2001;49:425–432.
- Gorno-Tempini ML, Hillis AE, Weintraub S, Kertesz A, Mendez M, Cappa SF, Ogar JM, Rohrer JD, Black S, Boeve BF, Manes F, Dronkers NF, Vandenberghe R, Rascovsky K, Patterson K, Miller BL, Knopman DS, Hodges JR, Mesulam MM, Grossman M: Classification of primary progressive aphasia and its variants. Neurology 2011;76:1006–1014.
- Hamaguchi T, Kitamoto T, Sato T, Mizusawa H, Nakamura Y, Noguchi M, Furukawa Y, Ishida C, Kuji I, Mitani K, Murayama S, Kohriyama T, Katayama S, Yamashita M, Yamamoto T, Udaka F, Kawakami A, Ihara Y, Nishinaka T, Kuroda S, Suzuki N, Shiga Y, Arai H, Maruyama M, Yamada M: Clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease. Neurology 2005;64:643–648.
- Tschampa HJ, Kallenberg K, Kretzschmar HA, Meissner B, Knauth M, Urbach H, Zerr I: Pattern of cortical changes in sporadic Creutzfeldt-Jakob disease. Am J Neuroradiol 2007;28:1114–1118.
- Meissner B, Kallenberg K, Sanchez-Juan P, Collie D, Summers DM, Almonti S, Collins SJ, Smith P, Cras P, Jansen GH, Brandel JP, Coulthart MB, Roberts H, Van Everbroeck B, Galanaud D, Mellina V, Will RG, Zerr I: MRI lesion profiles in sporadic Creutzfeldt-Jakob disease. Neurology 2009;72:1994–2001.
- Krasnianski A, Meissner B, Schulz-Schaeffer W, Kallenberg K, Bartl M, Heinemann U, Varges D, Kretzschmar HA, Zerr I: Clinical features and diagnosis of the MM2 cortical subtype of sporadic Creutzfeldt-Jakob disease. Arch Neurol 2006;63:876–880.
- Coulthart MB, Jansen GH, Olsen E, Godal DL, Connolly T, Chol BC, Wang Z, Cashman NR: Diagnostic accuracy of cerebrospinal fluid protein markers for sporadic Creutzfeldt-Jakob disease in Canada: a 6-year prospective study. BMC Neurol 2011;11:133.
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