An inquiry for the neuropathology community from two illustrious German neurologists regarding possible olivary dysgenesis

Prof. Dr. Gunther Deuschl

Prof. Dr. Olaf Jansen

 

 

The 

 

 

 

 

 

 

 

 

 

 

 

The following is an inquiry received from Prof. Dr. Günther Deuschl, Chairman of the Department of Neurology, UKSH, Christian-Albrechts University, Kiel, Germany. Please post your ideas on this case in the comments section:

Dear friends,

I have a patient presenting with  different noises: one subjectively in the thyroid region (nothing objectively), one in the region of the ear (coming erratically in groups, some resemblance to earclicks, again nothing objectively), one pulse synchronous noise (with a known neuroradiologically confirmed and treated fistula carotis to sinus cavernosus). I would have considered her to have only the latter clinical problem. All the other complaints seemed to be due to enhanced introspection without definite psychiatric diagnosis. Then the neuroradiologist (Prof. O. Jansen, Kiel University, Germany) made an MRI scan and found this curious ‘Tic-sign’ of the brain stem (you will understand when you look at the images below, which you can click on to enlarge). It shows definite circumscribed regional and bilateral atrophy of the inferior olive. Interestingly there seems to be some ‘white matter’ remaining. . Indeed an olivary agenesis or dysgenesis is one of the possibilities. The lady has no cerebellar signs as you see this sometimes (but not always) after olivary destruction.

I wonder if anybody has ever seen something like this and have an insights on a diagnosis?

Best regards for taking your time to read and see this.

Best regards,
Guenther