66 year old with progressive cognitive and neurological decline, becoming catatonic within 2 months.
MRI DWI [1, 2, 3] shows cortical ribboning (cortex diffusion restriction [1]), and diffusion restriction in the deep gray nuclei and insula [2, 3] in a symmetric fashion.
MRI FLAIR [4] shows abnormal bright signal at the sites of diffusion restriction.
MRI postcontrast T1 [5] and SWI [6] are normal.
Based on the above findings, there was a strong suspicion this was something bad. A lumbar puncture was performed under appropriate infection control guidelines. Results showed positive CSF results for 14-3-3, T-tau protein, and RT-QuIC. The patient had already been discharged to a nursing facility when these results came back, so the nursing facility was notified as well as the public health department. The patient was placed on comfort care.
Final diagnosis: sporadic Creutzfeldt-Jakob disease (CJD).
This disease would be called mad cow disease if you caught it from a cow. I would say it's one of the scarier neurodegenerative diseases because it's infectious too. In the CDC link I put in, they recommend wiping down the entire place after a procedure with 1 NORMAL sodium hydroxide. That's crazy strong, caustic stuff. Usually, surfaces touched by a patient or his/her blood/etc after a procedure are just wiped with your standard disinfectants.
I agree with you though. The neurodegenerative diseases are very scary. Insidious onset, soul-destroying, and no cure.