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

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Presentation transcript:

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

* Typical / Atypical * Diffusion

JH Hise Radiology 1996:

Young J. AJNR 2005 vol. 26 (6) pp mental decline /dementia ataxia, 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 protein Young G AJNR 2003 vol. 24 (8) pp. 1560

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

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

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

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

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

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

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

Ryutarou U RadioGraphics 2006; 26:S191–S weeks

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

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

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

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

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

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

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

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

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

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

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

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

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

Smith AB Radiographics 28 (7)

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

Case 1 bx proven Case 2 PCR for JC in CSF initial1 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,

* 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

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

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

DWI B=3000 DWI FA Initial 4 week after Tx

Smith AB Radiographics 28 (7) IRIS : immune reconstitution inflammatory syndrome

Smith AB Radiographics 28 (7)

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

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

Bag 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

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

* 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.

Review

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