BACKGROUND: Most cases of Creutzfeldt-Jakob disease (CJD) in recipients of human cadaveric growth hormone present with a cerebellar syndrome. Dementia is thought to occur late and as a minor feature of the illness. However, neuropsychology data published on these cases are largely qualitative and anecdotal. The first published case does include a neuropsychological assessment seven months after the onset of a cerebellar syndrome, showing evidence of intellectual decline. Subsequent reports hint that cognitive problems may be present in the initial stages of the illness. OBJECTIVE: To assess early cognition in Creutzfeldt-Jakob disease in recipients of pituitary derived human growth hormone. METHODS: Detailed neuropsychology assessment is reported at referral (mean 4.5 months from the onset of symptoms; range 4 to 6 months) in five patients with histologically proven human growth hormone derived CJD. RESULTS: All cases presented with a cerebellar syndrome and only one had noticed mild memory problems. On formal testing, however, four had demonstrable mild intellectual decline, as measured on the WAIS-R. One case showed selective visual memory impairment and frontal executive dysfunction. CONCLUSIONS: These findings suggest that, although not the presenting feature, mild cognitive decline may be evident in the early stages of CJD associated with human cadaveric growth hormone treatment.
BACKGROUND: Most cases of Creutzfeldt-Jakob disease (CJD) in recipients of human cadaveric growth hormone present with a cerebellar syndrome. Dementia is thought to occur late and as a minor feature of the illness. However, neuropsychology data published on these cases are largely qualitative and anecdotal. The first published case does include a neuropsychological assessment seven months after the onset of a cerebellar syndrome, showing evidence of intellectual decline. Subsequent reports hint that cognitive problems may be present in the initial stages of the illness. OBJECTIVE: To assess early cognition in Creutzfeldt-Jakob disease in recipients of pituitary derived humangrowth hormone. METHODS: Detailed neuropsychology assessment is reported at referral (mean 4.5 months from the onset of symptoms; range 4 to 6 months) in five patients with histologically proven humangrowth hormone derived CJD. RESULTS: All cases presented with a cerebellar syndrome and only one had noticed mild memory problems. On formal testing, however, four had demonstrable mild intellectual decline, as measured on the WAIS-R. One case showed selective visual memory impairment and frontal executive dysfunction. CONCLUSIONS: These findings suggest that, although not the presenting feature, mild cognitive decline may be evident in the early stages of CJD associated with human cadaveric growth hormone treatment.
Authors: C Bernoulli; J Siegfried; G Baumgartner; F Regli; T Rabinowicz; D C Gajdusek; C J Gibbs Journal: Lancet Date: 1977-02-26 Impact factor: 79.321
Authors: P Parchi; A Giese; S Capellari; P Brown; W Schulz-Schaeffer; O Windl; I Zerr; H Budka; N Kopp; P Piccardo; S Poser; A Rojiani; N Streichemberger; J Julien; C Vital; B Ghetti; P Gambetti; H Kretzschmar Journal: Ann Neurol Date: 1999-08 Impact factor: 10.422
Authors: P Brown; M Preece; J P Brandel; T Sato; L McShane; I Zerr; A Fletcher; R G Will; M Pocchiari; N R Cashman; J H d'Aignaux; L Cervenáková; J Fradkin; L B Schonberger; S J Collins Journal: Neurology Date: 2000-10-24 Impact factor: 9.910
Authors: Diana Caine; Renata J Tinelli; Harpreet Hyare; Enrico De Vita; Jessica Lowe; Ana Lukic; Andrew Thompson; Marie-Claire Porter; Lisa Cipolotti; Peter Rudge; John Collinge; Simon Mead Journal: Ann Clin Transl Neurol Date: 2015-04-07 Impact factor: 4.511