Cjdprespml
Download
1 / 65

CJDPRESPML - PowerPoint PPT Presentation


  • 111 Views
  • Uploaded on

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 ,

loader
I am the owner, or an agent authorized to act on behalf of the owner, of the copyrighted work described.
capcha
Download Presentation

PowerPoint Slideshow about ' CJDPRESPML' - janet


An Image/Link below is provided (as is) to download presentation

Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author.While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server.


- - - - - - - - - - - - - - - - - - - - - - - - - - E N D - - - - - - - - - - - - - - - - - - - - - - - - - -
Presentation Transcript
Cjdprespml

  • Creutzfeldt–Jakob diseasePosterior Reversible Encephalopathy SyndromeProgressive Multifocal Leukoencephalopathy

CJDPRESPML


Themes

THEMES


Cjd creutzfeldt jakob disease
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


Young G AJNR 2003 vol. 24 (8) pp. 1560


Normal

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


High b value

B =1000

B=3000

HighB value

H Hyare. AJNR 2010 vol. 31 (3) pp. 521


Cjd variant

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
Endstage CJD

BC Tzeng. AJNR 1997 vol. 18 (3) pp. 583


Differential dx
Differential Dx

Amogh N. Hegde, MD, FRCR RadioGraphics2011; 31:5–30


Japanese encephalitis
Japanese Encephalitis

Amogh N. Hegde, MD, FRCR RadioGraphics2011; 31:5–30



Causes

Causes

Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036


1 month

Follow up

Bartynski W. AJNR 2006 vol. 27 (10) pp. 2179


Covarrubias D. AJNR 2002 vol. 23 (6) pp. 1038


PRES

Covarrubias D. AJNR 2002 vol. 23 (6) pp. 1038


W atershed

watershed?

Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320


W atershed1

watershed?

Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320


3 typical patterns parieto occipital holohemispheric frontal sulcus

3 typical patternsParieto-occipitalHolohemisphericFrontal sulcus

Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320


Mild

Mckinny A. AJR 2007 vol. 189 (4) pp. 904


Moderate

Moderate

Mckinny A. AJR 2007 vol. 189 (4) pp. 904


Severe

Severe

Mckinny A. AJR 2007 vol. 189 (4) pp. 904


W Bartynski AJNR 2007 vol. 28 (7) pp. 1320


Tumefactive

Tumefactive

Mckinny A. AJR 2007 vol. 189 (4) pp. 904


Hemorrhagic

Hemorrhagic

Mckinny A. AJR 2007 vol. 189 (4) pp. 904


H Hefzy AJNR 2009 vol. 30 (7) pp. 1371


More blood

More Blood

Mckinny A. AJR 2007 vol. 189 (4) pp. 904


Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036


Follow up

Follow up

W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179


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
cPML - classic

Smith AB Radiographics 28 (7) 2033-58 2008


Aids dementia
AIDS dementia

Smith AB Radiographics 28 (7) 2033-58 2008


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


Pml pons

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,


Haart

  • 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
Treatment T1 Hyperintensity

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


High b value1

B=1000

B=3000

High B value

Usiskin SI AJNR 28 Feb 2007


Pml tx

DWI

FA

B=3000

Initial

DWI

FA

4 week after Tx

PML Tx


I pml inflammatory
iPML - inflammatory


IRIS : immune reconstitution inflammatory syndrome

Smith AB Radiographics 28 (7) 2033-58 2008


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

Other JC virus infections

Is PML still an adequate name?


Jc encephalitis

Cortical pyramidal cell infected

3 months

later

JC Encephalitis

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



Progression
Progression

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


Pml course

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
Intralesional cyst

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


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


Tysabri natalizumab

Tysabri (natalizumab)


Tysabri natalizumab1

  • 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 atypical cjd pres pml
Typical / Atypical matter involvement; frequent involvement of posterior fossa. CJDPRESPML

Review


END matter involvement; frequent involvement of posterior fossa.


sCJD matter involvement; frequent involvement of posterior fossa.

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


ad