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Comment & Response |

Creutzfeldt-Jakob Disease—Reply

Heather Angus-Leppan, MD, FRCP1; Peter Rudge, FRCP2; Simon Mead, PhD2; John Collinge, MD, FRCP, FRS2; Angela Vincent, FRCPath3
[+] 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.
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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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December 1, 2013
Felix F. Benninger, MD, PhD; Israel Steiner, MD
1Department of Neurology, Rabin Medical Center, Beilinson Hospital, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
JAMA Neurol. 2013;70(12):1588-1589. doi:10.1001/jamaneurol.2013.4412.
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