Creutzfeldt-Jakob disease (CJD) is a misnomer. In 1921, the Hamburg neuropathologist Alfons Maria Jakob (1884-1931) published three papers, “Über eigenartige Erkrankunbgen des Zentralnervensystems mit bemerkens- wertem anatomischen Befunde” (“On peculiar illnesses of the central nervous system with remarkable anatomical findings”)1,2,3.
In these and in a paper of 1923 (ref. 4), he described the cases of two men and three women aged between 34 and 51 who progressively developed disturbances of motor functions, speech and emotion, with obvious personality changes and loss of memory; eventually they were unable to move, stand or speak, and died in dementia between a few weeks and a year after the start of more serious symptoms.
In 1920, the Breslau neurologist Hans Gerhard Creutzfeldt (1885-1964) had described “a peculiar focal illness of the central nervous system” of the patient Berta E.5. Jakob writes in his second paper on these new diseases that he came to the conclusion that Creutzfeldt's case refers to a “nosologically very closely connected if not identical affection” (“nosologisch sehr nahestehende, wenn nicht wesensgleiche Affektion”) to his own cases6. Thus the designation “Creutzfeldt-Jakob-Krankheit” was logical. It had first been used in 1922 (ref. 7).
Medicine today considers CJD as a rare, progressive and always deadly transmissible spongiform encephalopathy (TSE), and also as a prion disease. Careful perusal of the case reported by Creutzfeldt on the basis of the clinical and pathological findings and his illustrations showed that his case does not belong to the TSE group.
E. E. Manuelidis reports: “Dr Creutzfeldt after the Second World War told me that his case did not bear any resemblance to the cases described by Jakob”8. Strictly speaking, therefore, the sickness should be called Jakob's disease. It was of course Jakob's own fault that his name is connected erroneously with Creutzfeldt.
But, even of Jakob's five cases, only two are what we understand today as CJD. C. L. Masters was able to re-examine the original slide preparations of Jakob's cases still preserved in the archives of the department of neurology at the University of Hamburg, and he found that only the third and fifth of Jakob's cases fall within the present diagnostic criteria. Five of six subsequent cases published from Jakob's laboratory, however, under his supervision, showed the typical changes of spongiform encephalopathy9.
Because of bovine spongiform encephalopathy, Creutzfeldt-Jakob disease has become almost a household name. It seems impossible, therefore, to drop the Creutzfeldt part of this term because everybody would think that Jakob's disease is something different. But it would be possible to do Jakob historical justice by changing the order of the two names so that his comes first, and by always using the term Jakob-Creutzfeldt disease as is often done in German medical literature.
Jakob, A. D. Z. Nervenheilk. 70, 132-146 (1921a).
Jakob, A. Z. ges. Neurol. Psychiatr. 64, 147-228 (1921b).
Jakob, A. Med. Klin. 17, 382-386 (1921c).
Jakob, A. Monogr. Gesamtgeb. Neurol. Psychiatr. 37, 215–345 (1923).
Creutzfeldt, H. G. Z. ges. Neurol. Psychiatr. 57, 1–18 (1920).
Jakob, A. Z. ges. Neurol. Psychiatr. 64, 174–175 (1921).
Spielmeyer, W. Klin. Wochenschr. 1, 1817–1819 (1922).
Manuelidis, E. E. J. Neuropathol. 44, 1–17 (1985).
Masters, C. L. & Gajdusek, D. C. in Recent advances in Neuropathology Vol. 2 (eds Smith, W. T. & Cavanagh, J. B.) 139-163 (Churchill Livingstone, Edinburgh, 1982).
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