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Creutzfeldt-Jakob disease and cerebral amyloid angiopathy

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Abstract

An 83-year-old female with no personal or familial neurological history developed progressive gait and speech disturbance and left motor deficit. She suffered intractable seizures and died 3 months after the onset of neurological signs. Neuropathology showed severe spongiosis and gliosis in the cortex and basal ganglia, and diffuse cerebral amyloid angiopathy. Immunostaining for prion protein (PrP) showed intense PrP positivity in areas of confluent spongiosis and some granular staining in astrocytes. The cortical vessel walls stained positively for β/A4 amyloid but not for PrP amyloid. Both types of amyloid were only observed in pericapillary parenchyma, in areas with severe spongiosis. There were only a few tangles and neuritic plaques in the temporal cortex; amyloid plaques were not present either by silver stains or immunostains. There was neither arteriopathic leukoencephalopathy nor cerebral hemorrhage. Immunoblot analysis of brain extracts revealed an abnormal proteinase K-resistant isoform of PrP. Association of Creutzfeldt-Jakob disease and Cerebral amyloid angiopathy in the absence of Alzheimer changes is unusual. The association of PrP and β/A4 amyloid deposits could have been fortuitous in an 83-year-old patient. An etiopathogenic relationship between β/A4 amyloid deposition and PrP accumulation may also be considered.

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Authors and Affiliations

  1. Département de Pathologie (Neuropathologie), Hôpital Henri Mondor, F-94010, Créteil cedex, France

    F. Gray

  2. Laboratoire d'Anatomie Pathologique (Neuropathologie), Hôpital Raymond Poincaré, Garches, Faculté de Médecine Paris-Ouest, France

    F. Gray & F. Chrétien

  3. Département de Neurosciences Médicales, Faculté de Médecine de Créteil, Université Paris-Val de Marne, France

    F. Gray, P. Cesaro & J. D. Degos

  4. Service de Biochimie et de Neurobiologie, Hôpital Saint Louis, Paris, France

    J. Chatelain, P. Beaudry & J. L. Laplanche

  5. Service Central d'Anatomie et de Cytologie Pathologiques, Hôpital Lariboisière, Faculté de Médecine Paris VII, France

    J. Mikol

  6. Neuropathology Laboratory, Western General Hospital, The University of Edinburgh, UK

    J. Bell

  7. Division of Neuropathology, Institute of Pathology, Case Western Reserve University, Cleveland, Ohio, USA

    P. Gambetti

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  1. F. Gray
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Supported by a Concerted Action of the European community This case was the subject of a preliminary presentation at the eighty-seventh meeting of the British Neuropathological Society [17]

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Gray, F., Chrétien, F., Cesaro, P. et al. Creutzfeldt-Jakob disease and cerebral amyloid angiopathy. Acta Neuropathol 88, 106–111 (1994). https://doi.org/10.1007/BF00294366

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  • DOI: https://doi.org/10.1007/BF00294366

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