Sporadic Creutzfeldt-Jakob disease (sCJD)
Rapidly progressing dementia
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Basal ganglia T1 and DWI hyperintensity. High cortical DWI signal.
3 case question available
This is certainly a tough case. There is considerable overlap in presentation between much of the differential, but a characteristic feature in this patient provides a clue to diagnosis. But first, let’s narrow down the differential of high basal ganglia T1 signal:
- Leigh syndrome is primarily seen in children
- osmotic demyelination syndrome is T2 hyperintense, and usually in putamen and external capsule
- Wilson disease can be distinguished based on the current disease trajectory
- if copper predominating, T1 hyperintense, T2 hypointense
- if gliosis predominates, T1 hypointense, T2 hyperintense
- hypoxic ischemic injury is more common in globus pallidus, but can affect all deep nuclei
The likely diagnosis is Creutzfeldt-Jakob disease (CJD).
- Bekiesińska-Figatowska, M., Kuczyńska-Zardzewiały, A., Pomianowska, B., Kajdana, K., Szpak, G. M., Iwanowska, B., & Mądzik, J. (2012). The value of magnetic resonance imaging in the early diagnosis of Creutzfeldt-Jakob disease - own experience. Polish journal of radiology / Polish Medical Society of Radiology, 77(1), 63–67.
- Carswell, C., Thompson, A., Lukic, A., Stevens, J., Rudge, P., Mead, S., Collinge, J., et al. (2012). MRI findings are often missed in the diagnosis of Creutzfeldt-Jakob disease. BMC Neurology, 12(1), 153. doi:10.1186/1471-2377-12-153
- Kumaran, S., Gupta, K., Pushpa, B., Viswamitra, S., & Joshy, E. (2012). Diffusion-weighted imaging: As the first diagnostic clue to Creutzfeldt Jacob disease. Journal of Neurosciences in Rural Practice, 3(3), 408. doi:10.4103/0976-3147.102645
- Satoh, K., Nakaoke, R., Nishiura, Y., Tsujino, A., Motomura, M., Yoshimura, T., Sasaki, K., et al. (2011). Early detection of sporadic CJD by diffusion-weighted MRI before the onset of symptoms. Journal of Neurology, Neurosurgery & Psychiatry, 82(8), 942–943. doi:10.1136/jnnp.2008.155242
- Vitali, P., Maccagnano, E., Caverzasi, E., Henry, R. G., Haman, A., Torres-Chae, C., Johnson, D. Y., et al. (2011). Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology, 76(20), 1711–1719. doi:10.1212/WNL.0b013e31821a4439