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A 30-year-old man died of a progressive neuropsychiatric illness of approximately 17 months' duration with a clinical picture strongly suggestive of variant Creutzfeldt–Jakob disease (vCJD).1 His initial symptoms, at the age of 28, were of personality change with anxiety and irritability. Lower abdominal and leg pain were troublesome early symptoms. At about 12 months of illness, he developed tremor and unsteadiness, leading to progressive walking problems. Increasing social withdrawal and behavioural problems were complicated by the development of progressive memory and general cognitive impairment. Visual hallucinations and paranoid delusions occurred in the later parts of his illness. During the course of his illness, he developed the following neurological signs: cognitive impairment, limb and gait cerebellar ataxia, mild dysarthria and mild pyramidal signs. Extensive neurological investigations revealed no cause other than prion disease, including consideration of a wide variety of inflammatory, neoplastic, immunological and neurodegenerative illnesses. The EEG showed diffuse slow activity without periodic complexes. The cerebral MRI showed changes suggestive of vCJD but did not show the characteristic pulvinar sign.2 No tonsil biopsy or neuropathological examination was performed. Sequencing of the PRNP gene showed …
Funding The CJD Surveillance Unit is funded by the Department of Health and the Scottish Home Office Department of Health.
Competing interests None.
Patient consent Obtained from the patient's family.
Ethics approval Ethics approval was provided by the Multi-Centre Research Ethics Committee for Scotland (REC reference number: 05/MRE00/67).
Provenance and peer review Not commissioned; externally peer reviewed.