Subject: Alzheimer-type neuropathology in a 28-year old patient with iatrogenic CJD after dural grafting
Date: March 9, 2007 at 9:15 am PST

HUMAN-04

Alzheimer-type neuropathology in a 28-year old patient with iatrogenic

Creutzfeldt-Jakob disease after dural grafting

M Preusser1, T Stroebel1, E Gelpi1, 2, M Eiler3, G Broessner4, E Schmutzhard4, H Budka1, 2

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, Austria

We report the autopsy case of a 28-year old male patient who had received a cadaverous dura

mater graft after a traumatic open skull fracture with tearing of 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 diseaseassociated

prion protein (PrPsc) in a synaptic pattern and western blot analysis identified PrPsc 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 1. the

iCJD case with the longest incubation time after dural grafting reported so far, 2. the youngest

documented patient with concomitant CJD and Alzheimer-type neuropathology to date, 3. the

first description of Alzheimer type-changes in iCJD, and 4. 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.


249 of 411 pages...tss


http://www.tse-forum.de/tse_forum/de...FINAL_nov2.pdf


TSS