NR AOPK
AU Cordery,R.J.; Hall,M.; Cipolotti,L.; al-Sarraj,S.; O'Donovan,D.G.; Davidson,L.; Adlard,P.; Rossor,M.N.
TI Early cognitive decline in Creutzfeldt-Jakob disease associated with human growth hormone treatment
QU Journal of Neurology, Neurosurgery and Psychiatry 2003 Oct; 74(10): 1412-6
IA http://jnnp.bmj.com/cgi/content/full/74/10/1412
PT journal article
AB 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.
MH Adult; Cadaver; Case Report; Cerebellum/*pathology; Cognition Disorders/*etiology; Creutzfeldt-Jakob Syndrome/*complications/etiology; Drug Contamination; Female; Human; Human Growth Hormone/*adverse effects; Male; Pituitary Gland
AD Dementia Research Group, Institute of Neurology, University College London, London WC1, UK
SP englisch
PO England