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Comment & Response
December 2013

Creutzfeldt-Jakob Disease—Reply

Author Affiliations
  • 1Department of Clinical Neurosciences, Royal Free Hospital, London, England
  • 2MRC Prion Unit, University College London Institute of Neurology, London, England
  • 3University of Oxford, Nuffield Department of Clinical Neurosciences, Oxford, England
JAMA Neurol. 2013;70(12):1589. doi:10.1001/jamaneurol.2013.4777

In Reply In response to our article,1 Benninger and Steiner describe an older man with rapid cognitive decline, personality change, ataxia, and autoantibodies. Serum and cerebrospinal fluid testing results were positive for Caspr-2 antibodies at low titer and immune treatments were given promptly, but the patient died within a month of admission. The diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) was supported by magnetic resonance imaging appearances of bilateral parietal-occipital hyperintensity with the cortical ribbon sign,2,3 which have sensitivity and specificity of 96% and 93%, respectively (Vitali et al2), although Zerr et al3 reported sensitivity of 83% and emphasized the importance of a multimodal diagnostic approach. The magnetic resonance imaging findings were in fact the only investigation in support of the diagnosis of sCJD in Benninger and Steiner’s patient. Cerebrospinal 14-3-3 results, with an overall sensitivity of 85% to 95%,4 were not reported; electroencephalography findings were nonspecifically slow, without the periodic complexes seen in two-thirds of cases usually late in the disorder.5 The diagnosis could not be proven in their case because brain biopsy and postmortem analysis were declined by the family.

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