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Medical Service, Edith Nourse Rogers Memorial VA Medical Center, Bedford (Dr. Mandell)
Braintree Rehabilitation Hospital, Braintree (Dr. Alexander)
Department of Neurology, Boston University School of Medicine, Boston, MA (Drs. Mandell and Alexander)
Department of Neurology- Neurosurgery, McGill University, and the Montreal Neurological Institute, Montreal, Quebec, Canada. (Dr. Carpenter)
Progressive aphasia without dementia (primary progressive aphasia) is increasingly recognized as an important neurobehavioral syndrome. Clinical diagnosis of progressive aphasia is difficult early in its course, and the differential diagnosis is usually said to include Alzheimer's and Pick's diseases. We report a 61-year-old man with autopsy-proven Creutzfeldt-Jakob disease (CJD) whose major initial manifestation was a progressive, fluent aphasia. Myoclonus was absent, and characteristic EEG abnormalities appeared relatively late. We believe that this case of CJD is unique in its presentation of profound and isolated aphasia. CJD should be considered in the differential diagnosis of the progressive aphasia syndrome.
Address correspondence and reprint requests to Dr. Mandell, Medical Service (111), Edith Nourse Rogers Memorial VAMC, 200 Springs Road, Bedford, MA 01730.
Presented in part at the thirty-ninth annual meeting of the American Academy of Neurology, New York, NY, April 1987.
Received March 3, 1987. Accepted for publication in final form June 6, 1988.
This article has been cited by other articles:
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  N. Kapur, P. Abbott, A. Lowman, and R. G. Will The neuropsychological profile associated with variant Creutzfeldt-Jakob disease Brain, December 1, 2003; 126(12): 2693 - 2702. [Abstract] [Full Text] [PDF]
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 J S Snowden, D M A Mann, and D Neary Distinct neuropsychological characteristics in Creutzfeldt-Jakob disease J. Neurol. Neurosurg. Psychiatry, December 1, 2002; 73(6): 686 - 694. [Abstract] [Full Text] [PDF]
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 A. Kertesz and D. G. Munoz Primary progressive aphasia: A review of the neourobiology of a common presentation of Pick complex American Journal of Alzheimer's Disease and Other Dementias, January 1, 2002; 17(1): 30 - 36. [Abstract] [PDF]
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J. D W Greene, J. R Hodges, J. W Ironside, and C. P Warlow Progressive aphasia with rapidly progressive dementia in a 49 year old woman J. Neurol. Neurosurg. Psychiatry, February 1, 1999; 66(2): 238 - 243. [Full Text]
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N. Nagaratnam and K. Nagaratnam Slowly progressive aphasia heralding dementia American Journal of Alzheimer's Disease and Other Dementias, November 1, 1997; 12(6): 245 - 251. [Abstract] [PDF]
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 C. Andersen, C. Dahl, O. Almkvist, P. Ostberg, P. Julin, and L.-O. Wahlund Bilateral Temporal Lobe Volume Reduction Parallels Cognitive Impairment in Progressive Aphasia Arch Neurol, October 1, 1997; 54(10): 1294 - 1299. [Abstract] [PDF]
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J. D. W. Greene, K. Patterson, J. Xuereb, and J. R. Hodges Alzheimer Disease and Nonfluent Progressive Aphasia Arch Neurol, October 1, 1996; 53(10): 1072 - 1078. [Abstract] [PDF]
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H. Karbe, A. Kertesz, and M. Polk Profiles of Language Impairment in Primary Progressive Aphasia Arch Neurol, February 1, 1993; 50(2): 193 - 201. [Abstract] [PDF]
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C. Confavreux, B. Croisile, P. Garassus, G. Aimard, and M. Trillet Progressive Amusia and Aprosody Arch Neurol, September 1, 1992; 49(9): 971 - 976. [Abstract] [PDF]
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