creutzfeldt-jakobdisease-160511045910 (1).pptx

creutzfeldt-jakobdisease-160511045910 (1).ppt

1. Creutzfeldt-Jakob disease Dr. Muhammad Bin Zulfiqar PGR IV FCPS Services Institute of Medical Sciences / Hospital [email protected]

2. CAse A 72-year-old male presented with ten days history of altered sensorium, involuntary movement of limbs, and progressive dementia. There was no history of fever or vomiting. On examination, the patient was disoriented, the muscle tone was increased, and tendon reflexes were exaggerated. The Babinski's sign was positive bilaterally. There were no signs of meningeal irritation or cerebellar signs. The EEG was normal..

3. Creutzfeldt-Jakob disease CJD is a transmissible, rapidly progressive, invariably fatal neurodegenerative disorder... It is caused by the accumulation of abnormal prion protein in the neurons resulting in their spongiform degeneration... MRI, especially the DWI sequence, plays a vital role in suggesting an early diagnosis... It characteristically shows symmetrical T2 hyperintensities with restricted diffusion in bilateral cerebral cortices and basal ganglia...

4. Creutzfeldt-Jakob disease Confirmation of the disease requires demonstration of periodic sharp wave complexes on EEG or 14-3-3 protein in CSF... The disease is transmissible and early diagnosis is helpful in appropriate patient management... The disease is fatal and no treatment is currently available.

5.  Diffusion weighted MRI sequence (A and B) shows symmetrical hyperintensity involving bilateral caudate nucleus, globus pallidus, putamen, and cerebral cortex (arrows). The FLAIR image (C) also shows diffuse hyperintensity involving the cerebral cortices and basal ganglia (arrows)

6. MRI hyper intensities in familial CJD. The figure shows scans from  three different patients with different mutations in PRNP. White arrows show  lesions with high signal intensity. 

7.  MRI shows symmetrical hyperintensities in the putamen and caudate head within two months seen on T2-weighted and FLAIR 

8. Case 54 years old man. Rapid cognitive impairment.

9. Hyperintensities seen in basal ganglia, thalami and cerebral cortices

10. Hyperintensities seen in basal ganglia, thalami and cerebral cortices

11. Hyperintensities seen in basal ganglia, thalami and cerebral cortices

12. MRI shows subtle abnormal signal intensity in the basal ganglion, particularly evident within the caudate nucleus. The basal ganglia are hyperintense on DWI (not shown, but hypointense on ADC) & slightly hyperintense on T2. As mentioned previously, this is more evident with regard to the caudate nucleus. There is no abnormal cortical signal or diffusion restriction. 

13. Take Home Message MR Imaging particularly diffusion weighted imaging is highly sensitive in diagnosis of Creutzfeldt-Jakob disease. Typical features are Hyperintense signals in basal ganglia Thalami Cerebral cortices

14. THANX