Coexistence of Creutzfeldt-Jakob disease, Lewy body disease, and Alzheimer's disease pathology: An autopsy case showing typical clinical features of Creutzfeldt-Jakob disease
Corresponding Author
Takashi Haraguchi
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Takashi Haraguchi, MD, PhD, Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, 4066 Hayashima-cho, 701-0304 Tsukubo-gun Okayama, Japan. Email: [email protected]Search for more papers by this authorSeishi Terada
Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Shikata-cho, Okayama,
Search for more papers by this authorHideki Ishizu
Department of Laboratory and Medicine, Zikei Institute, Urayasuhon-cho, Okayama, and
Search for more papers by this authorKenichi Sakai
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorYasuyuki Tanabe
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorTaiji Nagai
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorHiroshi Takata
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorKeigo Nobukuni
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorYuetsu Ihara
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorTetsuyuki Kitamoto
Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, Seiryou-cho, Sendai, Japan
Search for more papers by this authorShigetoshi Kuroda
Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Shikata-cho, Okayama,
Search for more papers by this authorCorresponding Author
Takashi Haraguchi
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Takashi Haraguchi, MD, PhD, Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, 4066 Hayashima-cho, 701-0304 Tsukubo-gun Okayama, Japan. Email: [email protected]Search for more papers by this authorSeishi Terada
Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Shikata-cho, Okayama,
Search for more papers by this authorHideki Ishizu
Department of Laboratory and Medicine, Zikei Institute, Urayasuhon-cho, Okayama, and
Search for more papers by this authorKenichi Sakai
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorYasuyuki Tanabe
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorTaiji Nagai
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorHiroshi Takata
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorKeigo Nobukuni
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorYuetsu Ihara
Department of Neurology, National Hospital Organization Minami-Okayama Medical Center, Hayashima-cho, Tsukubo-gun, Okayama,
Search for more papers by this authorTetsuyuki Kitamoto
Division of CJD Science and Technology, Department of Prion Research, Center for Translational and Advanced Animal Research on Human Diseases, Tohoku University Graduate School of Medicine, Seiryou-cho, Sendai, Japan
Search for more papers by this authorShigetoshi Kuroda
Department of Neuropsychiatry, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Shikata-cho, Okayama,
Search for more papers by this authorAbstract
We report here an autopsy case of sporadic Creutzfeldt-Jakob disease (CJD) without hereditary burden and with a clinical course typical of sporadic CJD. A 77-year old man developed memory disturbance, followed by gait disturbance and myoclonus. He died of bronchopneumonia 5 months after the disease onset. Post-mortem examination revealed neuronal loss, astrocytosis, and patchy spongiosis in the cerebral cortex and lenticular nuclei. Synaptic-type deposits of prion protein were present in the cerebral cortex. Additionally, Lewy bodies were observed in the cerebral cortex and substantia nigra. Furthermore, senile plaques compatible with definite Alzheimer's disease according to Consortium to Establish a Registry for Alzheimer's disease criteria and neurofibrillary changes of the limbic system consistent with Braak stage IV were found. Based on a review of the published literature, this autopsy case is very rare, and we suppose that the incidence of CJD accompanied by Lewy body disease and Alzheimer's disease is very low.
REFERENCES
- 1 Prusiner SB. Genetic and infectious prion diseases. Arch Neurol 1993; 50: 1129–1153.
- 2 DeArmond SJ, Kretzschmar HA, Prusiner SB. Prion disease. In: DI Graham, PL Lantos, eds. Greenfield's Neuropathology, 7th edn. London: Arnold, 2002; 273–323.
- 3 Budka H, Parchi P, Head MK et al. Sporadic Creutzfeldt-Jakob disease. In: D Dickson, ed. Pathology & Genetics: Neurodegenerative Diseases. Basel: ISN Neuropath Press, 2003; 287–297.
- 4 McKeith IG, Galasko D, Kosaka K et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): report of the consortium on DLB international workshop. Neurology 1996; 47: 1113–1124.
- 5 McKeith IG, Dickson DW, Lowe J et al. Diagnosis and management of dementia with Lewy bodies: third report of the DLB Consortium. Neurology 2005; 65: 1863–1872.
- 6 Lippa CF, Smith TW, Swearer JM. Alzheimer's disease and Lewy body disease: a comparative clinicopathological study. Ann Neurol 1994; 35: 81–88.
- 7 Gaches J, Supino-Viterbo V, Foncin JF. Association of Alzheimer's disease and Creutzfeldt-Jakob's disease. Acta Neurol Belg 1977; 77: 202–212.
- 8 Muramoto T, Kitamoto T, Koga H, Tateishi J. The coexistence of Alzheimer's disease and Creutzfeldt-Jakob disease in a patient with dementia of long duration. Acta Neuropathol (Berl) 1992; 84: 686–689.
- 9
Wakabayashi K,
Hinokura K,
Takahashi H,
Seki K,
Tanaka M,
Ikuta F.
Coexistence of Creutzfeldt-Jakob disease and senile dementia of the Alzheimer type.
Neuropathology
1995; 15: 122–126.
10.1111/j.1440-1789.1995.tb00254.x Google Scholar
- 10 Brown P, Jannotta F, Gibbs CJ Jr, Baron H, Guiroy DC, Gajdusek DC. Coexistence of Creutzfeldt-Jakob disease and Alzheimer's disease in the same patient. Neurology 1990; 40: 226–228.
- 11 Brown P, Gibbs CJ Jr, Rodgers-Johnson P et al. Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease. Ann Neurol 1994; 35: 513–529.
- 12 Hainfellner JA, Wanschitz J, Jellinger K, Liberski PP, Gullotta F, Budka H. Coexistence of Alzheimer-type neuropathology in Creutzfeldt-Jakob disease. Acta Neuropathol (Berl) 1998; 96: 116–122.
- 13 Hyman BT, Trojanowski JQ. Consensus recommendations for the postmortem diagnosis of Alzheimer disease from the National Institute on Aging and the Reagan Institute Working Group on diagnostic criteria for the neuropathological assessment of Alzheimer disease. J Neuropathol Exp Neurol 1997; 56: 1095–1097.
- 14 Mirra SS, Heyman A, McKeel D et al. The Consortium to Establish a Registry for Alzheimer's Disease (CERAD). Part II. Standardization of the neuropathologic assessment of Alzheimer's disease. Neurology 1991; 41: 479–486.
- 15 Parchi P, Castellani R, Capellari S et al. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996; 39: 767–778.
- 16 Braak H, Braak E. Neuropathological staging of Alzheimer-related changes. Acta Neuropathol (Berl) 1991; 82: 239–259.
- 17 Powers JM, Liu Y, Hair LS, Kascsack RJ, Lewis LD, Levy LA. Concomitant Creutzfeldt-Jakob and Alzheimer diseases. Acta Neuropathol (Berl) 1991; 83: 95–98.
- 18 Tsuchiya K, Yagishita S, Ikeda K et al. Coexistence of CJD and Alzheimer's disease: an autopsy case showing typical clinical features of CJD. Neuropathology 2004; 24: 46–55.
- 19 Amyot R, Gauther C Sur la maladie de Creutzfeldt Jakob. Deux observations anatomo-cliniques. Conception uniciste. Rev Neurol (Paris) 1964; 110: 473–488.
- 20 Hirano A, Ghatak NR, Johnson AB, Partnow MJ, Gomori AJ. Argentophilic plaques in Creutzfeldt-Jakob disease. Arch Neurol 1972; 26: 530–542.
- 21 Horoupian DS, Ross RT. Cucumber shaped and 35 nm particles in Creutzfeldt-Jakob disease. Can J Neurol Sci 1975; 2: 203–207.
- 22 Liberski PP, Papierz W, Alwasiak J. Creutzfeldt-Jakob disease with plaques and paired helical filaments. Acta Neurol Scand 1987; 76: 428–432.
- 23 Iida T, Doh-ura K, Kawashima T, Abe H, Iwaki T. An atypical case of sporadic Creutzfeldt-Jakob disease with Parkinson's disease. Neuropathology 2001; 21: 294–297.
- 24 Ezrin-Waters C, Resch L, Lang AE. Coexistence of idiopathic Parkinson's disease and Creutzfeldt-Jakob disease. Can J Neurol Sci 1985; 12: 272–273.
- 25 Vital A, Canron MH, Gil R, Hauw JJ, Vital C. A sporadic case of Creutzfeldt–Jakob disease with beta-amyloid deposits and alpha-synuclein inclusions. Neuropathology 2007; 27: 273–277.
- 26 Doh-ura K, Kitamoto T, Sakaki Y, Tateishi J. CJD discrepancy. Nature 1991; 353: 801–802.
- 27 Miyazono M, Kitamoto T, Doh-ura K, Iwaki T, Tateishi J. Creutzfeldt-Jakob disease with codon 129 polymorphism (valine): a comparative study of patients with codon 102 point mutation or without mutations. Acta Neuropathol (Berl) 1992; 84: 349–354.
- 28 Kosaka K. Diffuse Lewy body disease in Japan. J Neurol 1990; 237: 197–204.
- 29 Kosaka K. Diffuse Lewy body disease. Neuropathology 2000; 20 (Suppl): 21–35.
- 30 Yokota O, Tsuchiya K, Uchihara T et al. Lewy body variant of Alzheimer's disease or cerebral type Lewy body disease? Two autopsy cases of presenile onset with minimal involvement of the brainstem. Neuropathology 2007; 27: 21–35.
Citing Literature
