HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS REGISTER		Author Keyword(s) Vol Page [Advanced]

This Article Full Text Full Text (PDF) Submit a response Alert me when this article is cited Alert me when eLetters are posted Alert me if a correction is posted Citation Map Services Email this link to a friend Similar articles in this journal Similar articles in PubMed Add article to my folders Download to citation manager Request Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Preusser, M Articles by Budka, H Search for Related Content PubMed PubMed Citation Articles by Preusser, M Articles by Budka, H Related Collections New Variant Creutzfeld-Jakob Disease/BSE/Mad Cow Disease Other Neurology Dementia

Alzheimer-type neuropathology in a 28 year old patient with iatrogenic Creutzfeldt-Jakob disease after dural grafting

¹ Institute of Neurology, Medical University Vienna, Austria
² Austrian Reference Centre for Human Prion Diseases (OERPE), General Hospital Vienna, Austria
³ Department of Neurology, LKH Rankweil, Austria
⁴ Department of Neurology, Medical University Innsbruck, Innsbruck, Austria

Correspondence to:
Correspondence to:
Dr H Budka
Institute of Neurology, Medical University of Vienna, Waehringer Guertel 18-20, 4J, 1097 Vienna, Austria; herbert.budka{at}kin.at

ABSTRACT
We report the case of a 28 year old man who had received a cadaverous dura mater graft after a traumatic open skull fracture with tearing of the dura at the age of 5 years. A clinical suspicion of Creutzfeldt-Jakob disease (CJD) was confirmed by a brain biopsy 5 months prior to death and by autopsy, thus warranting the diagnosis of iatrogenic CJD (iCJD) according to WHO criteria. Immunohistochemistry showed widespread cortical depositions of disease associated prion protein (PrP^sc) in a synaptic pattern, and western blot analysis identified PrP^sc of type 2A according to Parchi et al. Surprisingly, we found Alzheimer-type senile plaques and cerebral amyloid angiopathy in widespread areas of the brain. Plaque-type and vascular amyloid was immunohistochemically identified as deposits of beta-A4 peptide. CERAD criteria for diagnosis of definite Alzheimer’s disease (AD) were met in the absence of neurofibrillar tangles or alpha-synuclein immunoreactive inclusions. There was no family history of AD, CJD, or any other neurological disease, and genetic analysis showed no disease specific mutations of the prion protein, presenilin 1 and 2, or amyloid precursor protein genes. This case represents (a) the iCJD case with the longest incubation time after dural grafting reported so far, (b) the youngest documented patient with concomitant CJD and Alzheimer-type neuropathology to date, (c) the first description of Alzheimer-type changes in iCJD, and (d) the second case of iCJD in Austria. Despite the young patient age, the Alzheimer-type changes may be an incidental finding, possibly related to the childhood trauma.

Keywords: Alzheimers disease; trauma; iatrogenic Creutzfeldt-Jakob disease

This article has been cited by other articles:

				R G Macfarlane, S J Wroe, J Collinge, T A Yousry, and H R Jager Neuroimaging findings in human prion disease J. Neurol. Neurosurg. Psychiatry, July 1, 2007; 78(7): 664 - 670. [Abstract] [Full Text] [PDF]

				P. Brown, J.-P. Brandel, M. Preese, and T. Sato Iatrogenic Creutzfeldt-Jakob disease: The waning of an era Neurology, August 8, 2006; 67(3): 389 - 393. [Abstract] [Full Text] [PDF]