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Brain, Vol. 113, No. 6, 1891-1909, 1990
© 1990 Guarantors of Brain

research-article

SPONGIFORM ENCEPHALOPATHY TRANSMITTED EXPERIMENTALLY FROM CREUTZFELDT-JAKOB AND FAMILIAL GERSTMANN-STRÄUSSLER-SCHEINKER DISEASES

H. F. BAKER1, L. W. DUCHEN2,, JEAN M. JACOBS2 and ROSALIND M. RIDLEY1

1Division of Psychiatry, MRC Clinical Research Centre Harrow, Middlesex 2Department of Neuropathology, Institute of Neurology, The National Hospital, Queen Square London, UK

Correspondence to: Correspondence to: Professor L. W. Duchen, Department of Neuropathology, Institute of Neurology, The National Hospital, Queen Square, London WC1N 3BG, UK.

A comparison was made of the effects of experimental intracerebral inoculation into marmosets of brain homogenates from a case of Creutzfeldt-Jakob disease (CJD) and from a member of the Wo. family with cerebral amyloid and spongiform encephalopathy-the Gerstmann-Straussler-Scheinker (GSS) syndrome. All the inoculated marmosets developed spongiform encephalopathy (SE) after incubation times of 20–23 months in the CJD group and 25–32 months in the GSS group. Subsequent passage from 1 affected animal in each group resulted in SE developing after 17 months incubation. In every animal inoculated with CJD or GSS material and in the 2 passage experiments the most severely affected region of the brain was the thalamus which in all cases was almost totally occupied by vacuoles. Other grey matter masses were less severely and less consistently affected. Vacuolation was observed in the cerebellar granule cell layer as well as in the molecular layer and the brain stem was finely vacuolated in all cases. There were only minor and inconsistent differences between the disease transmitted from CJD compared with GSS and some differences between the original transmissions and the SE caused by passaged inocula. Severe astrocytic gliosis accompanied the spongiform changes but no amyloid was identified in any of the marmosets with experimentally transmitted disease.

Received August 16, 1989. Revised December 28, 1989. Accepted January 9, 1990.


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