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Creutzfeldt – Jakob disease Posterior Reversible Encephalopathy Syndrome Progressive Multifocal Leukoencephalopathy. CJDPRESPML. Typical / Atypical Diffusion. THEMES. CJD - Creutzfeldt – Jakob disease. sCJD. JH Hise Radiology 1996:199 793-8. mental decline /dementia ataxia ,
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Creutzfeldt–Jakob diseasePosterior Reversible Encephalopathy SyndromeProgressive Multifocal Leukoencephalopathy CJDPRESPML
Typical / Atypical • Diffusion THEMES
sCJD JH Hise Radiology 1996:199 793-8
mental decline /dementia ataxia, sometimes visual disturbances Young J. AJNR 2005 vol. 26 (6) pp. 1551
sCJD – sporadic - 85% • fCJD – famial – 15% • Other less than 1% • vCJD – variant • Iatrogenic CJD Young G AJNR 2003 vol. 24 (8) pp. 1560
Definite – Path proven • Probable • EEG • CSF 14-3-3 protein Young G AJNR 2003 vol. 24 (8) pp. 1560
Kids normal BG bright Adults less from iron Normal Young J. AJNR 2005 vol. 26 (6) pp. 1551
40 yo Isolated Basal ganglia involvement 5% sCJD Young J. AJNR 2005 vol. 26 (6) pp. 1551
58% BG and cortex 35% Cortex 7% Neg B Messnier AJNR 2008 vol. 29 (8) pp. 1519
B =1000 B=3000 HighB value H Hyare. AJNR 2010 vol. 31 (3) pp. 521
Psychiatric and painful sensory symptoms CJD - variant Molloy S AJNR 2000 vol. 175 (2) pp. 55
vCJD D Collie AJNR 2003 vol. 24 (8) pp. 1560
vCJD D Collie AJNR 2003 vol. 24 (8) pp. 1560
fCJD Fulbright R. AJNR 2008 vol. 29 (9) pp. 1638
27 weeks RyutarouU RadioGraphics2006; 26:S191–S204
Endstage CJD BC Tzeng. AJNR 1997 vol. 18 (3) pp. 583
Differential Dx Amogh N. Hegde, MD, FRCR RadioGraphics2011; 31:5–30
Japanese Encephalitis Amogh N. Hegde, MD, FRCR RadioGraphics2011; 31:5–30
PRES Causes Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036
PRES 1 month Follow up Bartynski W. AJNR 2006 vol. 27 (10) pp. 2179
PRES Covarrubias D. AJNR 2002 vol. 23 (6) pp. 1038
PRES Covarrubias D. AJNR 2002 vol. 23 (6) pp. 1038
PRES watershed? Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320
PRES watershed? Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320
PRES 3 typical patternsParieto-occipitalHolohemisphericFrontal sulcus Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320
PRES Mild Mckinny A. AJR 2007 vol. 189 (4) pp. 904
PRES Moderate Mckinny A. AJR 2007 vol. 189 (4) pp. 904
PRES Severe Mckinny A. AJR 2007 vol. 189 (4) pp. 904
PRES W Bartynski AJNR 2007 vol. 28 (7) pp. 1320
PRES Tumefactive Mckinny A. AJR 2007 vol. 189 (4) pp. 904
PRES Hemorrhagic Mckinny A. AJR 2007 vol. 189 (4) pp. 904
PRES H Hefzy AJNR 2009 vol. 30 (7) pp. 1371
PRES More Blood Mckinny A. AJR 2007 vol. 189 (4) pp. 904
PRES Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036
PRES Follow up Follow up W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179
PRES W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179 11 day Follow up
W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179 SPECT HMPAO Tc-99m
cPML - classic Smith AB Radiographics 28 (7) 2033-58 2008
AIDS dementia Smith AB Radiographics 28 (7) 2033-58 2008
initial 1 month later Case 1 bx proven Normal 3 years prior PML Case 2 PCR for JC in CSF PML pons
5% of AIDS on autopsy • 90% die in 1 year without tx • PCR test for JC virus in CSF • JC virus infect oligodendrocytes,
PML without tx 4 months life expectancy • PML with tx increased the 1-year survival rate by 10%–50%. BerguiM,Neuroradiology2004;46:22–25 HAART Clifford DB Neurology 1999;52:623–25
Treatment T1 Hyperintensity Bag A. AJNR 2010 vol. 31 (9) pp. 1564
B=1000 B=3000 High B value Usiskin SI AJNR 28 Feb 2007
DWI FA B=3000 Initial DWI FA 4 week after Tx PML Tx