Bernd Scheithauer Has Died

A very sad letter from the Mayo Clinic received today:

Subject: Bernd Scheithauer

I just learned that Bernd Scheithauer, my treasured friend and colleague with whom we all had worked clinically or in the laboratory, was found dead at his home today.  He was not feeling well at work yesterday, and apparently died of natural causes at his home last evening or this morning.

Bernd was a rare individual and an academic giant.

Bernd had a deliciously naughty sense of humor and --with 24 indexed articles already in print this year-- I can just see him looking down on us, giggling, and saying, "Bill, take notice; publishing and perishing are not mutually exclusive."

I will certainly miss him.

Bill

William L. Lanier, M.D
Editor-in-Chief
Mayo Clinic Proceedings
Rochester, Minnesota  USA

Fusiform gyrus key to understanding neuroanatomic basis of Capgras delusion

Thanks to Doug Shevlin, MD (pictured with crawdaddy) for steering me toward this TED talk by neuroscientist Vilayanura Ramachandran, MD, PhD. Dr. Ramachandran discusses the neuroanatomical substrate of the Capgras delusion, a neurological deficit about which I have blogged before. Dr. Ramachandran explains that the origin of this fascinating deficit stems from a severing (typically resulting from a stroke or neurodegenerative disorder) of the connection between the fusiform gyrus (the face perception area of the brain) and the amygdala (which gages the emotional significance of a perception). Good stuff....  including the crawdaddy.

THE 5 HOTTEST TOPICS IN NEUROPATHOLGOY TODAY

By consensus, I present to you the five hottest topics in neuropathology today:

1. Chronic Traumatic Encephalopathy - There is an emerging recognition of CTE among those who have played contact sports. The elucidation of CTE will continue to have major public health policy implications.

2. Molecular Subtyping of Brain Tumors - For example, determination of the presence of O(6)-methylguanine DNA methyltransferase (MGMT) activity as a prognosticator of response to alkylating chemotherapy in gliomas is becoming increasingly important to our clinical neuro-oncology colleagues. Other molecular tests that are gaining popularity include IDH1 and EGFR. Molecular profile panels will become the standard of practice in the coming decades.

3. Brain Tumor Stem Cells - Questions about their existence and potential as targets for therapy have energized neuro-oncologic research.


4. Role of Microvascular Disease in Expression and Pathogenesis of Alzheimer Disease - The concept that Alzheimer disease may have a vascular pathogenesis may radically change the way the disease is prevented and treated.

5. Molecular Developments in Frontotemporal Lobar Degeneration -  As UCSF neurologist Bruce Miller, MD said: "Classification of FTLD is moving from a syndromic approach toward one based upon neuropathology and genetics." In particular, the description of the TDP-43 proteinopathies has had a major impact on our understanding of a previously unrecognized form of dementing disease.

There you have it. Thank you to everyone who contributed to this list. I think that it will help raise our collective eyes to the horizon.

What are the five hottest topics in neuropathology today?

What are the most promising or exciting topics in neuropathology today? Put up your list in the comments. Off the top of my head, in surgical neuropathology, the discussion regarding the utility of MGMT testing on high-grade gliomas (and whether PCR or IHC means of analysis) appears to be a timely topic. In non-surgical neuropathology, Chronic Traumatic Encephalopathy has been in the news lately. And what about TDP-43 protein in FTLD and MND? Come up with your own list and place in the comments. If we, as a virtual community, can come up with five of the most provocative topics in the field, I'll put them up on my next post.

"Coolest Picture Ever" of Baló’s Concentric Sclerosis

Dr. Mark Cohen, neuropathologist at Case Western Reserve University, sent me these "Images in Clinical Medicine" from the August 25, 2011 edition of the New England Journal of Medicine.  The accompanying text is as follows:

"A 25-year-old, left-handed man with known relapsing–remitting multiple sclerosis presented with a mild, nonfluent aphasic syndrome and left facial paresis that had developed within the previous 5 days. Magnetic resonance imaging (MRI) scans of the brain revealed a large lesion with a concentric-ring pattern consisting of alternating bands of higher and lower signal intensity (axial T2-weighted image above; sagittal image below). Although large in diameter, the lesion had only a minor mass effect. Baló’s concentric sclerosis, described in 1928 by Jószef Baló as a variant of acute multiple sclerosis, is histopathologically characterized by alternating layers of myelinated and demyelinated tissue. On MRI, Baló-like lesions are defined as two or more alternating bands of differing signal intensities. They may occur as solitary lesions, along with the plaques typically seen in multiple sclerosis, or (rarely) in the context of other disorders, such as neuromyelitis optica or viral infections of the central nervous system. The patient’s symptoms responded well within 1 week after the start of high-dose intravenous glucocorticoid therapy. On follow-up imaging 4 weeks later, both the size of the lesion and gadolinium enhancement had decreased."

Dr. Cohen's comment: "Coolest picture ever (if only it had path to go with it)." Agreed.