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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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CJDPRESPML

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  • Creutzfeldt–Jakob diseasePosterior Reversible Encephalopathy SyndromeProgressive Multifocal Leukoencephalopathy

CJDPRESPML


  • Typical / Atypical

  • Diffusion

THEMES


CJD - Creutzfeldt–Jakob disease


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 – Posterior Reversible Encephalopathy Syndrome


  • 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


PML – Progressive Multifocal Leukoencephalopathy


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


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


iPML - inflammatory


IRIS : immune reconstitution inflammatory syndrome

Smith AB Radiographics 28 (7) 2033-58 2008


Smith AB Radiographics 28 (7) 2033-58 2008


  • JCV granular cell neuronopathy– isolated cerebellar atrophy

  • JC meningitis – negative imaging

  • JC encephalitis – cortical involvement

Other JC virus infections

Is PML still an adequate name?


Cortical pyramidal cell infected

3 months

later

JC Encephalitis

Wuthrich C. Ann Neurol 2009;65:742– 48


JC solitary gray matter


Progression

Bag A. AJNR 2010 vol. 31 (9) pp. 1564


Initial – infarct?

1 month later, no Tx

On HAART 19 months

PML Course

Bag A. AJNR 2010 vol. 31 (9) pp. 1564


Bag A. AJNR 2010 vol. 31 (9) pp. 1564


Intralesional cyst

Bag A. AJNR 2010 vol. 31 (9) pp. 1564


Bag A. AJNR 2010 vol. 31 (9) pp. 1564


  • 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.

Tysabri (natalizumab)


Typical / AtypicalCJDPRESPML

Review


END


sCJD

Young G AJNR Am J Neuroradiol26:1551–1562, June/July 2005


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