Creutzfeldt jakob disease Dr. Muhammad Bin Zulfiqar
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In this presentation we will discuss the role of MRI in the diagnosis of this rare Prion disease. DWI is of diagnostic value in the diagnosis of Creutzfeldt Jakob Syndrome.
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Creutzfeldt jakob disease Dr. Muhammad Bin Zulfiqar
- 1. CREUTZFELDT-JAKOB DISEASE DR. MUHAMMAD BIN ZULFIQAR PGR IV FCPS SERVICES INSTITUTE OF MEDICAL SCIENCES / HOSPITAL RADIOMBZ@GMAIL.COM
- 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
- 15. • 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.
- 16. 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
- 17. THANX
Editor's Notes
- MRI done as a part of routine checkup showed diffuse hyperintensity in the cortex of bilateral frontal and parietal lobes and in bilateral caudate nuclei, globus pallidi, and putamina on diffusion weighted [DWI; Figure 1A and Figure 1B] and fluid-attenuated inversion recovery (FLAIR) images [Figure 1C] with restriction of diffusion. Imaging appearance was typical for CJD in the clinical setting. Subsequent CSF analysis for 14-3-3 protein was positive, confirming the diagnosis
- MRI done as a part of routine checkup showed diffuse hyperintensity in the cortex of bilateral frontal and parietal lobes and in bilateral caudate nuclei, globus pallidi, and putamina on diffusion weighted [DWI; Figure 1A and Figure 1B] and fluid-attenuated inversion recovery (FLAIR) images [Figure 1C] with restriction of diffusion... Imaging appearance was typical for CJD in the clinical setting... Subsequent CSF analysis for 14-3-3 protein was positive, confirming the diagnosis... Creutzfeldt-Jakob disease (CJD) is the most common member of a group of diseases called human transmissible spongiform encephalopathies (TSEs). Spongiform means that when viewed under a microcsope, a brain specimen from a CJD patient looks like a sponge (see photo below on right). Encephalopathy is a broadly defined term that means abnormal brain structure or function. Transmissable means that these diseases are contagious. However, they cannot be passed from one person to another through the air, by touching, or through other forms of casual contact. See the section on Transmission below for more information. TSEs are progressive disorders, which means that they get worse with time. Other members of the TSE group include Gertstmann-Sträussler-Scheinker disease (humans), kuru (humans), fatal familial insomnia (humans), scrapie (sheep, goats and mouflon), transmissible encephalopathy (mink), chronic wasting disease (deer and elk), and bovine spongiform encephalopathy (cows; BSE). As a group, prion disorders are extremely rare, with a major study of 11 countries around the world finding 1.67 annual deaths per million people (1). The same study found that deaths from CJD were 1.39 per million, which highlights the fact that CJD is the most common member of the group.