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* Creutzfeldt–Jakob disease Posterior Reversible Encephalopathy Syndrome Progressive Multifocal Leukoencephalopathy.

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Presentation on theme: "* Creutzfeldt–Jakob disease Posterior Reversible Encephalopathy Syndrome Progressive Multifocal Leukoencephalopathy."— Presentation transcript:

1 * Creutzfeldt–Jakob disease Posterior Reversible Encephalopathy Syndrome Progressive Multifocal Leukoencephalopathy

2 * Typical / Atypical * Diffusion

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4 JH Hise Radiology 1996:199 793-8

5 Young J. AJNR 2005 vol. 26 (6) pp. 1551 mental decline /dementia ataxia, sometimes visual disturbances

6 * sCJD – sporadic - 85% * fCJD – famial – 15% * Other less than 1% * vCJD – variant * Iatrogenic CJD Young G AJNR 2003 vol. 24 (8) pp. 1560

7 * Definite – Path proven * Probable * EEG * CSF 14-3-3 protein Young G AJNR 2003 vol. 24 (8) pp. 1560

8 Young J. AJNR 2005 vol. 26 (6) pp. 1551 Kids normal BG bright Adults less from iron

9 40 yo Young J. AJNR 2005 vol. 26 (6) pp. 1551 Isolated Basal ganglia involvement 5%

10 58% BG and cortex 35% Cortex 7% Neg B Messnier AJNR 2008 vol. 29 (8) pp. 1519

11 B =1000B=3000 H Hyare. AJNR 2010 vol. 31 (3) pp. 521

12 Molloy S AJNR 2000 vol. 175 (2) pp. 55 Psychiatric and painful sensory symptoms

13 D Collie AJNR 2003 vol. 24 (8) pp. 1560

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15 Fulbright R. AJNR 2008 vol. 29 (9) pp. 1638

16 Ryutarou U RadioGraphics 2006; 26:S191–S204 27 weeks

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

18 Amogh N. Hegde, MD, FRCR RadioGraphics 2011; 31:5–30

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

22 1 month Follow up Bartynski W. AJNR 2006 vol. 27 (10) pp. 2179

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

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

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

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

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

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

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

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

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

38 W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179 11 day Follow up

39 W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179 SPECT HMPAO Tc-99m

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41 Smith AB Radiographics 28 (7) 2033-58 2008

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

44 Case 1 bx proven Case 2 PCR for JC in CSF initial1 month later Normal 3 years prior PML

45 * 5% of AIDS on autopsy * 90% die in 1 year without tx * PCR test for JC virus in CSF * JC virus infect oligodendrocytes,

46 * 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

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

48 B=1000 B=3000 Usiskin SI AJNR 28 Feb 2007

49 DWI B=3000 DWI FA Initial 4 week after Tx

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51 Smith AB Radiographics 28 (7) 2033-58 2008 IRIS : immune reconstitution inflammatory syndrome

52 Smith AB Radiographics 28 (7) 2033-58 2008

53 * JCV granular cell neuronopathy– isolated cerebellar atrophy * JC meningitis – negative imaging * JC encephalitis – cortical involvement Is PML still an adequate name?

54 Wuthrich C. Ann Neurol 2009;65:742– 48 3 months later Cortical pyramidal cell infected

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

57 Initial – infarct? 1 month later, no Tx On HAART 19 months

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

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61 * 1) Progressive clinical disease. * 2) Typical MR imaging findings. * 3) Demonstration of JCV DNA in the CSF.

62 * 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.

63 Review

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65 Young G AJNR Am J Neuroradiol 26:1551–1562, June/July 2005


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