creutzfeldt jakob disease dr. muhammad bin zulfiqar
TRANSCRIPT
CREUTZFELDT-JAKOB DISEASE
DR. MUHAMMAD BIN ZULFIQAR
PGR IV FCPS SERVICES INSTITUTE OF MEDICAL SCIENCES / HOSPITAL
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..
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...
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.
• 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
• Hyperintensities seen in basal ganglia, thalami and cerebral
cortices
• Hyperintensities seen in basal ganglia, thalami and cerebral
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