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* CreutzfeldtJakob disease Posterior Reversible Encephalopathy Syndrome Progressive Multifocal Leukoencephalopathy Slide 2 * Typical / Atypical * Diffusion Slide 3 Slide 4 JH Hise Radiology 1996:199 793-8 Slide 5 Young J. AJNR 2005 vol. 26 (6) pp. 1551 mental decline /dementia ataxia, sometimes visual disturbances Slide 6 * sCJD sporadic - 85% * fCJD famial 15% * Other less than 1% * vCJD variant * Iatrogenic CJD Young G AJNR 2003 vol. 24 (8) pp. 1560 Slide 7 * Definite Path proven * Probable * EEG * CSF 14-3-3 protein Young G AJNR 2003 vol. 24 (8) pp. 1560 Slide 8 Young J. AJNR 2005 vol. 26 (6) pp. 1551 Kids normal BG bright Adults less from iron Slide 9 40 yo Young J. AJNR 2005 vol. 26 (6) pp. 1551 Isolated Basal ganglia involvement 5% Slide 10 58% BG and cortex 35% Cortex 7% Neg B Messnier AJNR 2008 vol. 29 (8) pp. 1519 Slide 11 B =1000B=3000 H Hyare. AJNR 2010 vol. 31 (3) pp. 521 Slide 12 Molloy S AJNR 2000 vol. 175 (2) pp. 55 Psychiatric and painful sensory symptoms Slide 13 D Collie AJNR 2003 vol. 24 (8) pp. 1560 Slide 14 Slide 15 Fulbright R. AJNR 2008 vol. 29 (9) pp. 1638 Slide 16 Ryutarou U RadioGraphics 2006; 26:S191S204 27 weeks Slide 17 BC Tzeng. AJNR 1997 vol. 18 (3) pp. 583 Slide 18 Amogh N. Hegde, MD, FRCR RadioGraphics 2011; 31:530 Slide 19 Slide 20 Slide 21 Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036 Slide 22 1 month Follow up Bartynski W. AJNR 2006 vol. 27 (10) pp. 2179 Slide 23 Covarrubias D. AJNR 2002 vol. 23 (6) pp. 1038 Slide 24 Slide 25 Bartynski W. AJNR 2007 vol. 28 (7) pp. 1320 Slide 26 Slide 27 Slide 28 Mckinny A. AJR 2007 vol. 189 (4) pp. 904 Slide 29 Slide 30 Slide 31 W Bartynski AJNR 2007 vol. 28 (7) pp. 1320 Slide 32 Mckinny A. AJR 2007 vol. 189 (4) pp. 904 Slide 33 Slide 34 H Hefzy AJNR 2009 vol. 30 (7) pp. 1371 Slide 35 Mckinny A. AJR 2007 vol. 189 (4) pp. 904 Slide 36 Bartynski W. AJNR 2008 vol. 29 (6) pp. 1036 Slide 37 W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179 Follow up Slide 38 W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179 11 day Follow up Slide 39 W Bartynsk AJNR 2006 vol. 27 (10) pp. 2179 SPECT HMPAO Tc-99m Slide 40 Slide 41 Smith AB Radiographics 28 (7) 2033-58 2008 Slide 42 Slide 43 Bag A. AJNR 2010 vol. 31 (9) pp. 1564 Slide 44 Case 1 bx proven Case 2 PCR for JC in CSF initial1 month later Normal 3 years prior PML Slide 45 * 5% of AIDS on autopsy * 90% die in 1 year without tx * PCR test for JC virus in CSF * JC virus infect oligodendrocytes, Slide 46 * PML without tx 4 months life expectancy * PML with tx increased the 1-year survival rate by 10%50%. Bergui M,Neuroradiology 2004;46:2225 Clifford DB Neurology 1999;52:62325 Slide 47 Bag A. AJNR 2010 vol. 31 (9) pp. 1564 Slide 48 B=1000 B=3000 Usiskin SI AJNR 28 Feb 2007 Slide 49 DWI B=3000 DWI FA Initial 4 week after Tx Slide 50 Slide 51 Smith AB Radiographics 28 (7) 2033-58 2008 IRIS : immune reconstitution inflammatory syndrome Slide 52 Smith AB Radiographics 28 (7) 2033-58 2008 Slide 53 * JCV granular cell neuronopathy isolated cerebellar atrophy * JC meningitis negative imaging * JC encephalitis cortical involvement Is PML still an adequate name? Slide 54 Wuthrich C. Ann Neurol 2009;65:742 48 3 months later Cortical pyramidal cell infected Slide 55 Slide 56 Bag A. AJNR 2010 vol. 31 (9) pp. 1564 Slide 57 Initial infarct? 1 month later, no Tx On HAART 19 months Slide 58 Bag A. AJNR 2010 vol. 31 (9) pp. 1564 Slide 59 Slide 60 Slide 61 * 1) Progressive clinical disease. * 2) Typical MR imaging findings. * 3) Demonstration of JCV DNA in the CSF. Slide 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. Slide 63 Review Slide 64 Slide 65 Young G AJNR Am J Neuroradiol 26:15511562, June/July 2005