MRI and clinical syndrome in dura mater-related Creutzfeldt-Jakob disease.
OBJECTIVE
Iatrogenic Creutzfeldt-Jakob disease (iCJD) is mainly associated with dura mater (DM) grafts and administration of human growth hormones (hGH). Data on disease course in DM-CJD are limited. We describe the clinical and diagnostic findings in this patient group with special emphasis on MRI signal alterations.


METHODS
Ten DM-CJD patients were studied for their clinical symptoms and diagnostic findings. The MRIs were evaluated for signal increase of the cortical and subcortical structures.


RESULTS
DM-CJD patients had a median incubation time of 18 years and median disease duration of 7 months. The majority of patients were MM homozygous at codon 129 of the prion protein gene (PRNP) and presented with gait ataxia and psychiatric symptoms. No correlation between the graft site and the initial disease course was found. The MRI showed cortical and basal ganglia signal increase each in eight out of ten patients and thalamic hyperintensity in five out of ten cases. Of interest, patients with thalamic signal increase were homozygous for methionine.


CONCLUSIONS
The MRI findings in DM-CJD largely resemble those seen in sporadic CJD, as the cortex and basal ganglia are mainly affected.
Meissner
B
B
National TSE Reference Center, Dept. of Neurology, University of Göttingen, Robert-Koch-Str. 40, 37075, Göttingen, Germany. epicjd@med.uni-goettingen.de
Kallenberg
K
K
Sanchez-Juan
P
P
Ramljak
S
S
Krasnianski
A
A
Heinemann
U
U
Eigenbrod
S
S
Gelpi
E
E
Barsic
B
B
Kretzschmar
H A
HA
Schulz-Schaeffer
W J
WJ
Knauth
M
M
Zerr
I
I
D000328:Adult; D000368:Aged; D001259:Ataxia / Q000150:complications; D001921:Brain / Q000473:pathology*; D016380:Brain Tissue Transplantation / Q000009:adverse effects*; D007562:Creutzfeldt-Jakob Syndrome / Q000150:complications* / Q000175:diagnosis / Q000473:pathology*; D004252:DNA Mutational Analysis; D004388:Dura Mater / Q000637:transplantation*; D005260:Female; D006801:Humans; D007049:Iatrogenic Disease; D055256:Infectious Disease Incubation Period; D008279:Magnetic Resonance Imaging; D008297:Male; D001523:Mental Disorders / Q000150:complications; D008875:Middle Aged; D000072002:Prion Proteins; D011328:Prions / Q000235:genetics; D013997:Time Factors
C512656:PRNP protein, human; D000072002:Prion Proteins; D011328:Prions