CJDPRESPML
description
Transcript of CJDPRESPML
*CJDPRESPML
*Creutzfeldt–Jakob disease
Posterior Reversible Encephalopathy Syndrome
Progressive Multifocal Leukoencephalopathy
*THEMES
*Typical / Atypical
*Diffusion
*CJD - Creutzfeldt–Jakob
disease
*sCJDJH Hise Radiology 1996:199 793-8
Young J. AJNR 2005 vol. 26 (6) pp. 1551
mental decline /dementiaataxia, sometimes visual disturbances
*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
*Normal
Young J. AJNR 2005 vol. 26 (6) pp. 1551
Kids normal BG brightAdults less from iron
*sCJD
40 yo
Young J. AJNR 2005 vol. 26 (6) pp. 1551
Isolated Basal ganglia involvement 5%
58%BG and cortex
35% Cortex
7%Neg
B Messnier AJNR 2008 vol. 29 (8) pp. 1519
*HighB value
B =1000 B=3000
H Hyare. AJNR 2010 vol. 31 (3) pp. 521
*CJD - variantMolloy S AJNR 2000 vol. 175 (2) pp. 55
Psychiatric and painful sensory symptoms
*vCJDD Collie AJNR 2003 vol. 24 (8) pp. 1560
*vCJDD Collie AJNR 2003 vol. 24 (8) pp. 1560
*fCJDFulbright R. AJNR 2008 vol. 29 (9) pp. 1638
Ryutarou U RadioGraphics 2006; 26:S191–S204
27 weeks
*Endstage CJDBC Tzeng. AJNR 1997 vol. 18 (3) pp. 583
*Differential DxAmogh N. Hegde, MD, FRCR RadioGraphics 2011; 31:5–30
*Japanese EncephalitisAmogh N. Hegde, MD, FRCR RadioGraphics 2011; 31:5–30
*PRES – Posterior Reversible
Encephalopathy Syndrome
*Causes
*PRES
Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036
*PRES
1 monthFollow up
Bartynski W. AJNR 2006 vol. 27 (10) pp. 2179
Covarrubias D. AJNR 2002 vol. 23 (6) pp. 1038
*PRES
*PRES
Covarrubias D. AJNR 2002 vol. 23 (6) pp. 1038
*watershed?
*PRES
Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320
*watershed?
*PRES
Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320
* 3 typical patternsParieto-occipitalHolohemispheric
Frontal sulcus
*PRES
Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320
*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
*PRES
W Bartynski AJNR 2007 vol. 28 (7) pp. 1320
*TumefactiveMckinny A. AJR 2007 vol. 189 (4) pp. 904
*PRES
*Hemorrhagic
Mckinny A. AJR 2007 vol. 189 (4) pp. 904
*PRES
*PRES
H Hefzy AJNR 2009 vol. 30 (7) pp. 1371
*More Blood
Mckinny A. AJR 2007 vol. 189 (4) pp. 904
*PRES
*PRES
Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036
W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179
Follow up Follow up
*PRES
*PRES
W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179
11 dayFollow up
W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179
SPECT HMPAO Tc-99m
*PML – Progressive
Multifocal Leukoencephalop
athy
*cPML - classic
Smith AB Radiographics 28 (7) 2033-58 2008
*AIDS dementia
Smith AB Radiographics 28 (7) 2033-58 2008
Bag A. AJNR 2010 vol. 31 (9) pp. 1564
*PML pons
Case 1 bx proven
Case 2 PCR for JC in CSF
initial 1 month later
Normal 3 years prior PML
*5% of AIDS on autopsy
*90% die in 1 year without tx
*PCR test for JC virus in CSF
*JC virus infect oligodendrocytes,
*HAART
*PML without tx 4 months life expectancy
*PML with tx increased the 1-year survival rate by 10%–50%.
Bergui M,Neuroradiology 2004;46:22–25
Clifford DB Neurology 1999;52:623–25
*Treatment T1 HyperintensityBag A. AJNR 2010 vol. 31 (9) pp. 1564
*High B value
B=1000 B=3000
Usiskin SI AJNR 28 Feb 2007
*PML Tx
DWI B=3000
DWI
FA
FA
Initial
4 week after Tx
*iPML - inflammatory
Smith AB Radiographics 28 (7) 2033-58 2008
IRIS : immune reconstitution inflammatory syndrome
Smith AB Radiographics 28 (7) 2033-58 2008
*Other JC virus infections
*JCV granular cell neuronopathy– isolated cerebellar atrophy
*JC meningitis – negative imaging
*JC encephalitis – cortical involvement
Is PML still an adequate name?
*JC EncephalitisWuthrich C. Ann Neurol 2009;65:742– 48
3 monthslater
Cortical pyramidal cell infected
*JC solitary gray matter
*ProgressionBag A. AJNR 2010 vol. 31 (9) pp. 1564
*PML CourseBag A. AJNR 2010 vol. 31 (9) pp. 1564
Initial – infarct?
1 month later, no Tx
On HAART 19 months
Bag A. AJNR 2010 vol. 31 (9) pp. 1564
*Intralesional cystBag A. AJNR 2010 vol. 31 (9) pp. 1564
Bag A. AJNR 2010 vol. 31 (9) pp. 1564
*Tysabri (natalizumab)
*1) Progressive clinical disease.
*2) Typical MR imaging findings.
*3) Demonstration of JCV DNA in the CSF.
*Tysabri (natalizumab)
*1) Diffuse subcortical rather than periventricular white matter involvement; frequent involvement of posterior fossa.
*2) Irregular ill-defined infiltrating edge confined to the white matter.
*3) Persistent progression of the lesion confined within the white matter tract.
*4) No mass effect even in large lesions.
*5) Diffuse increased T2 signal intensity; recently involved areas more T2 hyperintense than the old areas.
*6) Initially iso- to hypointense with an incremental drop of
*T1 signal intensity with time; signal intensity never returning to normal.
*7) Typically no enhancement, even in large lesions.
*Typical / Atypical
CJD
PRES
PMLReview
*END
*sCJD Young G AJNR Am J Neuroradiol 26:1551–1562, June/July 2005