Early cognitive decline in Creutzfeldt-Jakob disease associated with human 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 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.
Dementia Research Group, Institute of Neurology, University College London, London WC1, UK.
R J
RJ
Cordery
M
M
Hall
L
L
Cipolotti
S
S
Al-Sarraj
D G
DG
O'Donovan
L
L
Davidson
P
P
Adlard
M N
MN
Rossor
D000328:Adult; D002102:Cadaver; D002531:Cerebellum / Q000473:pathology*; D003072:Cognition Disorders / Q000209:etiology*; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000209:etiology; D004340:Drug Contamination; D005260:Female; D019382:Human Growth Hormone / Q000009:adverse effects*; D006801:Humans; D008297:Male; D010902:Pituitary Gland
D019382:Human Growth Hormone