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Transmissible Spongiform Encephalopathy (Creutzfeldt-Jakob Disease): Atypical Clinical and Pathological Findings

Transmissible Spongiform Encephalopathy (Creutzfeldt-Jakob Disease): Atypical Clinical and... Abstract • A middle-aged neurosurgeon had an 18-month illness characterized by abnormal sleep patterns, paresthesias, and necrotizing cutaneous lesions with vasculitis and signs of cerebral, brainstem, vestibulocerebellar, and progressive spinal cord involvement. Biopsy specimens of nerve and skin showed an acute vasculitis with endovascular cellular proliferation in the pattern of a Köhlmeier-Degos lesion and focal epidermal necrosis. Mental changes and cranial-nerve signs developed. Myoclonus occurred occasionally during sleep. Akinetic mutism ensued. At autopsy, major abnormalities were limited to the nervous system and skin. Spongiform encephalopathy typical of Creutzfeldt-Jakob disease was found with amyloid kuru plaques. A cribriform change distinct from the spongiform change was seen focally in the white matter. Scarred skin lesions and a healed, partially obliterative arteritis were noted. Inoculation of brain and lung into nonhuman primates resulted in a spongiform encephalopathy. References 1. Hobson JA: Dreaming sleep attacks and desynchronized sleep enhancement: Report of a case of brainstem signs . Arch Gen Psychiatry 1975;32:1421-1424.Crossref 2. Gajdusek DC, Gibbs CJ Jr, Earle K, et al: Transmission of subacute spongiform encephalopathy to the chimpanzee and squirrel monkey from a patient with papulosis atrophicans maligna of Köhlmeier-Degos, in Subirana A, Burrows JM (eds): Proceedings of the Tenth International Congress of Neurology. Amsterdam, Excerpta Medica, 1974, International Congress Series No. 319, pp 390-392. 3. McFarland HR, Wood WG, Drowns BV, et al: Papulosis atrophicans maligna (Köhlmeier-Degos disease): A disseminated occlusive vasculopathy . Ann Neurol 1978;3:388-392.Crossref 4. De Reuck J, de Coster W, Otte G, et al: Papovavirus-like particles in a nigral type of Creutzfeldt-Jakob disease . J Neurol 1976;213:179-188.Crossref 5. Gibbs CJ Jr, Gajdusek DC: Atypical viruses as the cause of sporadic, epidemic and familial chronic diseases in man: Slow viruses and human diseases , in Pollard M (ed): Perspectives in Virology . New York, Raven Press, 1978, vol 10, pp 161-198. 6. Masters CL, Richardson EP Jr: Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): The nature and progression of spongiform change . Brain 1978;101:333-344.Crossref 7. Tateishi J, Ohta M, Koga M, et al: Transmission of chronic spongiform encephalopathy with kuru plaques from humans to small rodents . Ann Neurol 1979;5:581-584.Crossref 8. Masters CL, Gajdusek DC, Gibbs CJ Jr: The Gerstmann-Sträussler syndrome and the various forms of amyloid plaques which occur in the transmissible spongiform encephalopathies . Brain , to be published. 9. Bunge RP, Settlage PH: Neurological lesions in cats following cerebrospinal fluid manipulation . J Neuropathol Exp Neurol 1957;16:471-491.Crossref 10. Breuer PC, Pitman SW, Dawson DM, et al: Paraparesis following intrathecal cytosine arabinoside: A case report with neuropathologic findings . Cancer 1977;40:2817-2822.Crossref 11. Masters CL, Harris JO, Gajdusek DC, et al: Creutzfeldt-Jakob disease: Patterns of world-wide occurrence and the significance of familial and sporadic clustering . Ann Neurol 1979;5:177-188.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Neurology American Medical Association

Transmissible Spongiform Encephalopathy (Creutzfeldt-Jakob Disease): Atypical Clinical and Pathological Findings

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Publisher
American Medical Association
Copyright
Copyright © 1981 American Medical Association. All Rights Reserved.
ISSN
0003-9942
eISSN
1538-3687
DOI
10.1001/archneur.1981.00510080035002
Publisher site
See Article on Publisher Site

Abstract

Abstract • A middle-aged neurosurgeon had an 18-month illness characterized by abnormal sleep patterns, paresthesias, and necrotizing cutaneous lesions with vasculitis and signs of cerebral, brainstem, vestibulocerebellar, and progressive spinal cord involvement. Biopsy specimens of nerve and skin showed an acute vasculitis with endovascular cellular proliferation in the pattern of a Köhlmeier-Degos lesion and focal epidermal necrosis. Mental changes and cranial-nerve signs developed. Myoclonus occurred occasionally during sleep. Akinetic mutism ensued. At autopsy, major abnormalities were limited to the nervous system and skin. Spongiform encephalopathy typical of Creutzfeldt-Jakob disease was found with amyloid kuru plaques. A cribriform change distinct from the spongiform change was seen focally in the white matter. Scarred skin lesions and a healed, partially obliterative arteritis were noted. Inoculation of brain and lung into nonhuman primates resulted in a spongiform encephalopathy. References 1. Hobson JA: Dreaming sleep attacks and desynchronized sleep enhancement: Report of a case of brainstem signs . Arch Gen Psychiatry 1975;32:1421-1424.Crossref 2. Gajdusek DC, Gibbs CJ Jr, Earle K, et al: Transmission of subacute spongiform encephalopathy to the chimpanzee and squirrel monkey from a patient with papulosis atrophicans maligna of Köhlmeier-Degos, in Subirana A, Burrows JM (eds): Proceedings of the Tenth International Congress of Neurology. Amsterdam, Excerpta Medica, 1974, International Congress Series No. 319, pp 390-392. 3. McFarland HR, Wood WG, Drowns BV, et al: Papulosis atrophicans maligna (Köhlmeier-Degos disease): A disseminated occlusive vasculopathy . Ann Neurol 1978;3:388-392.Crossref 4. De Reuck J, de Coster W, Otte G, et al: Papovavirus-like particles in a nigral type of Creutzfeldt-Jakob disease . J Neurol 1976;213:179-188.Crossref 5. Gibbs CJ Jr, Gajdusek DC: Atypical viruses as the cause of sporadic, epidemic and familial chronic diseases in man: Slow viruses and human diseases , in Pollard M (ed): Perspectives in Virology . New York, Raven Press, 1978, vol 10, pp 161-198. 6. Masters CL, Richardson EP Jr: Subacute spongiform encephalopathy (Creutzfeldt-Jakob disease): The nature and progression of spongiform change . Brain 1978;101:333-344.Crossref 7. Tateishi J, Ohta M, Koga M, et al: Transmission of chronic spongiform encephalopathy with kuru plaques from humans to small rodents . Ann Neurol 1979;5:581-584.Crossref 8. Masters CL, Gajdusek DC, Gibbs CJ Jr: The Gerstmann-Sträussler syndrome and the various forms of amyloid plaques which occur in the transmissible spongiform encephalopathies . Brain , to be published. 9. Bunge RP, Settlage PH: Neurological lesions in cats following cerebrospinal fluid manipulation . J Neuropathol Exp Neurol 1957;16:471-491.Crossref 10. Breuer PC, Pitman SW, Dawson DM, et al: Paraparesis following intrathecal cytosine arabinoside: A case report with neuropathologic findings . Cancer 1977;40:2817-2822.Crossref 11. Masters CL, Harris JO, Gajdusek DC, et al: Creutzfeldt-Jakob disease: Patterns of world-wide occurrence and the significance of familial and sporadic clustering . Ann Neurol 1979;5:177-188.Crossref

Journal

Archives of NeurologyAmerican Medical Association

Published: Aug 1, 1981

References