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Sporadic Creutzfeldt–Jakob disease with cerebellar ataxia at onset in the UK
  1. S A Cooper1,
  2. K L Murray2,
  3. C A Heath2,
  4. R G Will2,
  5. R S G Knight2
  1. 1Formerly the National CJD Surveillance Unit, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK
  2. 2The National Creutzfeldt–Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, UK
  1. Correspondence to:
 S A Cooper
 Institute of Neurological Sciences, Southern General Hospital, 1345 Govan Road, Glasgow, UK; sarah.cooper{at}


Objective: To determine the frequency, in the UK, of sporadic Creutzfeldt–Jakob Disease (sCJD) with a cerebellar ataxic onset, and to describe the clinical features of the syndrome.

Methods: A retrospective review of autopsy-proved cases of sCJD cases in the UK, 1990–2005, identifying those presenting with cerebellar features without early cognitive decline.

Results: 29 of 618 (5%) patients with sCJD had an isolated cerebellar onset. Mean illness duration was 9 months. Subsequently, 21 (72%) developed myoclonus and 23 (79%) developed pyramidal features. Magnetic resonance imaging showed high signal in the basal ganglia in 11 of 14 (79%) patients. 7 of 15 (47%) patients were valine homozygotic at prion protein gene (PRNP)-129. Only 8 (28%) cases were referred to the surveillance unit after death.

Conclusion: A better definition of sCJD presenting with an isolated cerebellar syndrome might improve future case recognition and contribute to the determination of its cause.

  • CJD, Creutzfeldt–Jakob disease
  • CSF, cerebrospinal fluid
  • EEG, electroencephalogram
  • MRI, magnetic resonance imaging
  • NCJDSU, National CJD Surveillance Unit
  • PRNP, Prion protein gene
  • sCJD, sporadic Creutzfeldt–Jakob disease

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  • Published Online First 11 July 2006

  • Competing interests: None.