Creutzfeldt jakob disease Dr. Muhammad Bin Zulfiqar

CREUTZFELDT-JAKOB DISEASE

DR. MUHAMMAD BIN ZULFIQAR

PGR IV FCPS SERVICES INSTITUTE OF MEDICAL SCIENCES / HOSPITAL

[email protected]

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..


• 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...


• 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.

• 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)

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.

• MRI shows symmetrical hyperintensities in the putamen and caudate

head within two months seen on T2-weighted and FLAIR

CASE

• 54 years old man.

• Rapid cognitive impairment.

• Hyperintensities seen in basal ganglia, thalami and cerebral

cortices


cortices


cortices

• 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.

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

THANX

Health & Medicine

Creutzfeldt jakob disease Dr. Muhammad Bin Zulfiqar