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Early cognitive decline in Creutzfeldt-Jakob disease associated with human growth hormone treatment
  1. R J Cordery1,
  2. M Hall2,
  3. L Cipolotti2,
  4. S Al-Sarraj3,
  5. D G O’Donovan4,
  6. L Davidson5,
  7. P Adlard5,
  8. M N Rossor1
  1. 1Dementia Research Group, Institute of Neurology, University College London and Division of Neuroscience and Psychological Medicine, Faculty of Medicine, Imperial College of Science, Technology and Medicine, London WC1, UK
  2. 2Department of Neuropsychology, The National Hospital for Neurology and Neurosurgery, London WC1, UK
  3. 3Department of Neuropathology, Institute of Psychiatry, King’s College London, London SE5, UK
  4. 4Department of Neuropathology, Addenbrooke’s Hospital, Cambridge, UK
  5. 5Biochemistry, Endocrine and Metabolism Unit, Institute of Child Health, London WC1, UK
  1. Correspondence to:
 Professor Martin Rossor
 Dementia Research Group, The National Hospital for Neurology and Neurosurgery, Queen Square, London WC1N 3BG, UK;


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.

  • Creutzfeldt-Jakob disease
  • human growth hormone
  • neuropsychology

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  • Competing interests: none declared