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Brian S. Appleby, MD; Kristin K. Appleby, MD; Barbara J. Crain, MD, PhD; Peter V. Rabins, MD, MPH; Chiadi U. Onyike, MD, MHS; Mitchell T. Wallin, MD, MPH

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In reply

We thank Dr Barash for his comments regarding the data presented in our article.¹ While we are in strong agreement with Dr Barash, we want to ensure that readers are aware that the affective cases in our study are not cases of vCJD. Nine of the 13 cases (69.2%) underwent postmortem examination and did not have neuropathological or prion protein strain characteristics typical of vCJD.² Thus, all neuropathologically confirmed cases in our article were of the sCJD type.

In regard to Dr Barash's comments, expanding our knowledge of the various clinical presentations of prion diseases will improve surveillance of prion diseases in general. Information regarding the typical features of vCJD, including young age at onset (second to third decade of . . . [Full Text of this Article]

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Identification of Creutzfeldt-Jakob Disease Variants
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Characteristics of Established and Proposed Sporadic Creutzfeldt-Jakob Disease Variants
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