*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