Case 9 10/29/2018. CJD (Creutzfeldt -Jakob Disease) CJD (Creutzfeldt -Jakob Disease) CJD (Creutzfeldt -Jakob Disease)

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1 CJD (Creutzfeldt -Jakob Disease) Rare fatal neurodegen dz caused by infectious protein Prion (lacks nucleic acid)- causes spongiform changes of the brain and neuronal death. 4 types: scjd- 85% of cases Genetic CJD icjd- from transplant e.g. cornea, dura vcjd- mad cow; pulvinar sign on mri CJD (Creutzfeldt -Jakob Disease) scjd- Diagnostic Criteria: Definite- path dx Probable- progressive dementia with at least 2 of 4 clinical features: 1.myoclonus, 2. visual or cerebellar disturb, 3. pyramidal/ extrapyr dysfn, 4. akinetic mutism Periodic sharp and slow wave complexes in EEG proteins in csf and clinical duration < 2 years Possible- Clinical as above No PSWC in EEG No in CSF Duration < 2 years CJD (Creutzfeldt -Jakob Disease) MR scjd DWI- cortical gyriform > 3 gyri sparing white matter. Frontal, limbic, temporal, parietal, occ. Usu restricted dwi. Caudate and striatum freq involved, thalami ; may be unilat or bilat. FLAIR and T2 may be nl early on. No swelling Radiological assessment or CJD; Eur Radiol (2007) 17: Case 9 1

2 Gliomatosis Cerebri Diffuse: Take Home Points WHO: malignant neuroepithelial neoplasm of uncertain origin; most postulate it is astrocytic glioma usu starts low grade. Infiltrative neoplasm involving 2 or more lobes. Differentiate from multifocal glioma which is separated by normal parenchyma. DWI variable usu intermediate without restriction. No enhancement til it becomes hi grade Survival 48% 1 year, 27 % 3 years? role of chemo and RT. Many overlapping imaging features of stroke, herpes enceph, limbic enceph, status epilepticus, CJD, gliomatosis If crosses vasc boundaries (PCA line) think away from stroke If crosses vasc boundaries posteriorly favor MELAS If DWI not restricted think glioma If DWI involves cortex and striatum has no swelling and spares white matter think CJD Herpes and limbic enceph virtually indistinguishable When in doubt mention Herpes! Interrogate DWI images including skull base 2

3 Focal- Infection vs Tumor Bilat occipital masses Abscesses 3

4 Cystic mets Lung ca Role of DWI in Ddx Intracranial Cystic Mass Lesions Husain, M et al Surgical Neurology lesions DWI restriction differentiating abscess from non-abscess: Sensitivity 96% Specificity 96% PPV 98% NPV 92% False Neg in Mucormycosis False Pos in GBM 4

5 Hemorrhage mimics abscess Left sided weakness Stroke Protocol 5

6 Coma 6

7 Draining Ear & Coma Teaching Point Mass in temporal lobe or cerebellum make sure to check the mastoids! Pyogenic Abscess vs Tumor lz Favor Abscess DWI- Central Restricted Wall enhancementsmooth Perfusion- not elevated Favor Tumor DWI- Central not restricte Wall enhancementthick and irregular Perfusion- elevated 7

8 lz lz lz lz Lz Toxoplasmosis 8

9 Teaching Point Parasites may be negative on DWI 37 y.o. female from Mexico came with sudden onset of AMS, became comatose with dilated pupils 9

10 Endoscopic treatment of intraventricular lesions 10

11 Neurocyst 2 43 yo female Dizzy n/v 11

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13 Part 2: Focal- Summary Ring enhancing mass with central restricted diffusion should be considered abscess until proven otherwise Parasites can be DWI negative, positive or equivocal DWI may help you pick up small abscesses or parasitic lesions that are not readily identified on other sequences Lack of elevated perfusion favors abscess Colloid cyst 6 month h/o dizziness and tinnitus 13

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