Summary
An autopsy case of Creutzfeld-Jacob disease with widespread amyloid plaques is reported. A 45-year-old Japanese man, whose father had died of a similar disease, had a 5-year illness characterized by progressive cerebellar signs. Mental changes and brain-stem signs developed in the late stage. Myoclonus frequently occurred. Akinetic mutism ensued. The autopsy revealed spongiform encephalopathy with widespread amyloid plaques and extensive degeneration of the white matter. This disease, Western Gerstmann-Sträussler-Scheinker disease and panencephalopathic type of Creutzfeld-Jacob disease are discussed.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Adam J, Crow TJ, Duchen LW, Scaravilli F, Spikes E (1982) Familial cerebral amyloidosis and spongiform encephalopathy. J Neurol Neurosurg Psychiatry 45:37–45
Boellaard JW, Schlote W (1980) Subacute spongiforme Encephalopathie mit multiformer Plaquebildung. Eigenartige familiär-hereditäre Krankheit des Zentralnervensystems (spino-cerebellare Atrophie mit Demenz, Plaques and plaqueähnlichen Ablagerungen im Klein- und Grosshirn) (Gerstmann-Sträussler-Scheinker) Acta Neuropathol (Berl) 49:205–212
Brownell B, Oppenheimer DR (1965) An atactic form of subacute presenile polioencephalopathy (Creutzfeldt-Jacob disease). J Neurol Neurosurg Psychiatry 28:350–361
Bruce ME, Fraser H (1981) Effect of route of infection on scrapie mice. Neuropathol Appl Neurobiol 7:289–298
Chou SM, Martin JD (1971) Kuru-like plaques in a case of Creutzfelt-Jacob disease. Acta Neuropathol (Berl) 17:150–155
Gerstmann J, Sträussler E, Scheinker I (1936) Über eine eigenartige hereditär-familiäre Erkrankung des Zentralnervensystems. Zugleich ein Beitrag zur Frage des vorzeitigen lokalen Alterns. Z Neurol 154:736–762
Jellinger K, Heiss WD, Deiseenhammer E (1974) The ataxic form (cerebellar) of Creutzfeldt-Jacob disease. J Neurol 207:298–305
Kuzuhara S, Kanazawa I, Sasaki H, Nakanishi T, Shimamura K (1983) Gerstmann-Sträussler-Scheinker's disease. Ann Neurol 14:216–225
Krucke W, Beck E, Vitzhum HG (1973) Creutzfeldt-Jacob disease. Some unusual morphological features reminiscent of kuru. J Neurol 206:1–24
Masters CL, Gajdusek DC, Gibbs CL (1981) Creutzfeldt-Jacob disease. Virus isolation from the Gerstmann-Sträussler syndrome with an analysis of the various forms of amyloid plaue deposition in the virus-induced spongiform encephalopathy. Brain 104:559–588
Mizutani T, Okumura A, Oda M, Shiraki H (1981) Panencephalopathic type of Creutzfeldt-Jacob disease; primary involvement of the cerebral white matter. J. Neurol Neurosurg Psychiatry 44:103–115
Mizutani T (1981) Neuropathology of Creutzfeldt-Jacob disease in Japan with special reference to the panencephalopathic type. Acta Pathol Jpn 31:903–922
Peiffer J (1982) Gerstmann-Sträussler disease, atypical multiple sclerosis and carcinomas in a family of sheepbreeders. Acta Neuropathol (Berl) 56:87–92
Rosenthal NP, Keesey J, Crandall B, Brown J (1976) Familial neurological disease with spongiform encephalopathy. Arch Neurol 33:252–259
Schlote W, Boellaard JW, Schumm F, Stohr M (1980) Gerstmann-Sträussler-Scheinker disease. Electron-microscopic observation on a brain biopsy. Acta Neuropathol Berl) 52:203–211
Schumm F, Boellaard JW, Schlote W, Storh M (1981) Morbus Gerstmann-Sträussler-Scheinker. Familie Sch. — Ein Bericht über drei Kranke. Arch Psychiatr Nervenkr 230:179–196
Sung-Park T, Kleinman GM, Richardson P (1980) Creutzfeldt-Jacob disease with extensive degeneration of white matter. Acta Neuropathol (Berl) 52:239–242
Tateishi J, Satoh Y, Doi H, Koga M, Ohta M (1980) Experimental transmission of human subacute spongiform encephalopathy to small rodents. I. Clinical and histological observations. Acta Neuropathol (Berl) 51:127–134
Tateishi J, Sato Y, Nagara H, Boellaard JW (1984) Experimental transmission of classical Gerstmann-Sträussler-Scheinker disease. Neuropathol 5:234 (in Japanese)
Yagishita S (1981) Creutzfeldt-Jacob disease with kuru-like plaques in Japan. Acta Pathol Jpn 31:923–942
Yagishita S, Itoh Y, Nakazima S, Nakano T, Amano N (1984) The fine structure of amyloid plaques in Creutzfeld-Jacob disease. J Clin Electron Microsc 17:281–288
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Yagishita, S., Iwabuchi, K., Amano, N. et al. Further observation of Japanese Creutzfeldt-Jacob disease with widespread amyloid plaques. J Neurol 236, 145–148 (1989). https://doi.org/10.1007/BF00314329
Received:
Revised:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00314329