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The early diagnosis and management of Creutzfeldt-Jakob disease.

Cordery, R.J.; (2004) The early diagnosis and management of Creutzfeldt-Jakob disease. Doctoral thesis , University of London. Green open access

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This thesis describes work undertaken to improve the early diagnosis of variant Creutzfeldt-Jakob disease (vCJD), using existing clinical and research tools. Twenty-one cases referred to the National Hospital for Neurology and Neurosurgery and St. Mary's Hospital, London with suspected vCJD completed participation in the study. Fifteen cases were confirmed with definite or probable vCJD and six were given alternative diagnoses. These six cases with alternative diagnoses formed a control group. Further controls were recruited from patients referred with sporadic and familial forms of prion disease. A neuropsychiatry questionnaire comprising a battery of standardised tests was formulated. Of those with definite or probable vCJD, 86% exhibited anxiety, 93% irritability, 64% agitation and 79% displayed evidence of severe depressive symptoms. Fifty seven percent experienced simple delusions, most commonly of theft and suspicion and 36% described misidentifications (mean 8 months from illness onset). Behavioural change was common to all cases, 79% with aggression, 71% emotional lability and 79% sleep problems. Comprehensive neuropsychology assessments from those with vCJD were compared with sporadic and familial cases. Moderate to severe intellectual decline is characteristic of vCJD and impairment affects all cognitive domains. Only a minority of the vCJD cases presented with perceptual impairment compared with 50% of sporadic and familial cases. The proportion of cases with nominal impairment in the familial disease group was significantly lower than in the variant and sporadic groups. Serial volumetric MR imaging was only possible in a subgroup of cases with familial CJD. The annual mean rate of whole brain atrophy was 2.05% compared to 0.25% in normal controls. Single voxel proton magnetic spectroscopy performed in three cases with vCJD showed a 2.5 fold (150%) increase in the mean myo-inositol concentration and 50% reduction in N-acetylaspartate in the pulvinar region. Similar changes were seen in the caudate nucleus where no signal change was detected on T2 weighted images. The key to early diagnosis still relies on a high index of suspicion for vCJD and early referral to the appropriate specialist services. First hand experience of the problems faced by patients prompted a second, parallel project to be undertaken. A survey was conducted of all UK consultant neurologists and old age psychiatrists to assess current practices in the diagnosis and management of young people with dementia. It was concluded that young people may be under investigated if managed solely by an old age psychiatrist and may not receive adequate follow up services if managed solely by a neurologist.

Type: Thesis (Doctoral)
Title: The early diagnosis and management of Creutzfeldt-Jakob disease.
Identifier: PQ ETD:602513
Open access status: An open access version is available from UCL Discovery
Language: English
Additional information: Thesis digitised by Proquest
UCL classification: UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/1446588
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