Short report
New variant Creutzfeldt-Jakob disease: three case reports from
Leicestershire
H Allroggen, G Dennis, R J Abbott, I F Pye
Department of
Neurology, Leicester Royal Infirmary, Leicester, UK
Correspondence to: Dr H Allroggen, Queen Elizabeth Neuroscience Centre, University Hospital, Edgebaston, Birmingham B15 2TH, UK
Received 25 May 1999 and in revised form 20 August 1999;
Accepted 9 November
1999
Since a report in 1996 of 10 cases of Creutzfeldt-Jakob disease
(CJD) with onset in a younger than usual age, a pattern of the disease
has emerged. This includes early neuropsychiatric features and sensory
symptoms and neurological signs such as ataxia and involuntary
movements later in the course of the disease. Three patients with
varied clinical presentations and disease course seen at a single
neurology unit are described. The first patient was characterised by
cognitive and psychiatric symptoms together with neurological signs.
The second patient presented with unusual behavioural disturbance and
episodes of collapse. The third patient exhibited striking psychomotor
retardation and had abnormal CSF and MRI findings. All patients
succumbed in a state of akinetic mutism and myoclonus. All three
patients had the methionine/methionine genotype at codon 129 of the PrP
gene and in two of the three patients a tonsil biopsy was performed with positive results. These two patients also tested positive for the
14.3.3. protein in the CSF.
Whereas late features of the disease seem very similar in all cases,
the initial presentation was variable and underlines the uncertainty of
the range of the clinical phenotype. Successful diagnosis demands a
high index of clinical suspicion.
Keywords: new variant Creutzfeldt-Jakob disease; clinical phenotype; tonsil biopsy
© 2000 by Journal of Neurology, Neurosurgery, and Psychiatry
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