Monday, January 5, 2015

Dr. Pierluigi Gambetti steps down as director of national prion surveillance center

Pierluigi Gambetti, MD
Dr. Pierluigi Gambetti has stepped down as director of the National Prion Disease Pathology Surveillance Center. In a letter to members of the American Association of Neuropathologists, Dr. Gambetti writes:

"I will be resigning as Director of the National Prion Disease Pathology Surveillance Center effective January 1, 2015. I will continue to be associated with the Center in an advisory position and as consultant for special cases. Dr. Jiri Safar, Associate Professor of Pathology and Neurology at Case Western Reserve University, will be the new director."

Dr. Gambetti goes on to state that the NPDPSC "will remain unchanged", continuing to coordinate autopsies on suspected prion disease cases. Dr. Mark Cohen will continue to have primary responsibility for the histologic and immunohistochemical assessment of cases.

Friday, December 19, 2014

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

Prof. Dr. Gunther Deuschl
Prof. Dr. Olaf Jansen















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,



Tuesday, December 16, 2014

The Einstein/Rorke-Adams Connection

Dr. Lucy Rorke-Adams
I'm not sure how many in the neuropathology community know this, but our renowned colleague Lucy Rorke-Adams donated slices of Albert Einstein's brain to the Mütter Museum and Historical Medical Library in Philadelphia. Rorke-Adams, a senior neuropathologist at the Children's Hospital of Philadelphia, donated the 46 slices of brain tissue to the museum in 2011. Rorke-Adams had received the slides as a gift from local doctor, who in turn had bequeathed them from his colleague, a neuropathologist who examined the slides on behalf of Thomas Harvey, the man who removed Einstein's brain during an autopsy in 1955. Guess where my first stop will be on my next visit to Philly?

Monday, December 1, 2014

Best Post of September 2014: New ISN book on Peripherap Nerve Disorders coming soon

The next in our "Best of the Month Series is from September 26, 2014. The update on this post is that the book is NOW AVAILABLE!  The cost is a reasonable £119.11 (€148.80). (Don't ask me what that means in greenbacks!)

New ISN book – Peripheral Nerve Disorders – coming soon

A new book covering peripheral nerve diseases (part of the International Society of Neuropathology series)  is about to be released . The book editors are Jean-Michel Vallet and Joachim Weiss and includes chapters from a range of other international experts who have produced a clinically orientated guide to the pathology of peripheral nerve disorders. The book includes the latest molecular and pathological findings to provide the most up-to-date understanding of the pathogenesis of these disorders.

Tuesday, November 18, 2014

Robin Williams had Lewy Body Disease

Robin Williams
The official cause of Robin Williams' death, released Friday by the Marin County coroner, was ruled a suicide by hanging, with no evidence of alcohol or illegal drugs in his system and only therapeutic concentrations of prescribed medications.

Williams had long battled alcoholism, drug addiction and depression, but in November 2013 he was diagnosed with Parkinson's disease, according to his widow, after noticing a tremor in his left arm and difficulty moving on his left side as early as 2011.

Now a redacted pathology report from the autopsy on Williams' body has been made public and mentions "Diffuse Lewy body dementia,". Given that Dementia with Lewy Bodies can involve vivid visual hallucinations, it has now been speculated that perhaps such hallucinations may have lead to Williams' death.

Media reports, quoting anonymous "family sources," state that Williams' family believes that Lewy body disease was a critical "triggering" factor in his suicide. If so, this would be an unusual manifestation of the disease. Suicides have not been linked specifically to the hallucinations of Lewy Body Disease in the past.

Dennis Dickson, MD
"The use of the term dementia in the neuropathology report should not be inferred to mean that dementia was observed during life," warns Dennis Dickson of the Mayo Clinic in Jacksonville, who says he reviewed the neuropathology report.

"Mr. Williams was given a clinical diagnosis of (Parkinson's) and treated for motor symptoms. The report confirms he experienced depression, anxiety and paranoia, which may occur in either Parkinson's disease or dementia with Lewy bodies," Dickson said.

Thursday, October 30, 2014

"Atlas of Gross Neuropathology" to be released in the United States tomorrow!

I just received this nice email in my inbox this afternoon from a rising star in the neuropathology firmament. I hope that  readers will share their feedback on this new atlas in the comments section of Neuropathology Blog in the coming months:

Dear Brian:

I hope all is well for you.

I just wanted to let you know that our book is finally going to be released in the US tomorrow.

We would love to hear some feedback from people about the book. We hope it is useful in many settings, specialties and different levels of training and practice.

Very best wishes,

Sandra Camelo-Piragua, M.D.
Clinical Assistant Professor
Neuropathology Division
Pathology Department
University of Michigan

1301 Catherine St, MSB1  M4213
Ann Arbor, MI 48104
Phone: 734-936-1889
Fax:       734-615-2965

Friday, October 10, 2014

Best Post of August 2015: 35-year-old woman who died after a presumed seizure episode

The next in our "Best of the Month" series is from Thursday, August 21, 2014:

A 35-year-old woman died after a witness saw her "foaming at the mouth". A seizure was presumed to have occurred by emergency personnel. At autopsy, there was mild dilatation of the lateral and third ventricles:

This translucent mass was identified in the third ventricle:

Histological examination of the cyst contents revealed:

Although no ciliated epithelium was identified within the specimen, there were columnar cells with a pseudostratified configuration:

However, the majority of the cyst lining was flattened, simple epithelium:

Diagnosis is colloid cyst of the third ventricle. Normally one would see ciliated columnar cells lining the cyst, but they appear to be attenuated and flattened by the internal pressure of the cyst itself.