Best Post of April 2019: Progressive Multifocal Leukoencephalopathy in patients without obvious immunosuppression

The next in our "Best of the Month" series comes from Friday, April 12, 2019:

I recently received a case in consultation which turned out to be progressive multifocal leukoencephalopathy (PML). Reading the clinical history, it was not entirely clear what predisposed the patient to PML. It wasn't clear, that is, until my mentor (the illustrious BK DeMasters) referred me to a nine-year-old paper by Sarah Gheuens, Gerald Pierone, Patrick Peeters, and Igor J. Koralnik entitled Progressive Multifocal Leukoencephalopathy in individuals with minimal or occult immunosuppression (J Neurol Neurosurg Psychiatry2010;81:247-254). In this series, hepatic cirrhosis -- which was what my patient had -- was among the more common conditions in PML patients with minimal immunosuppression. Other conditions that can be associated with PML in the minimally immunosuppressed are those with renal failure and idiopathic CD4+ lymphopenia,

The authors discuss the possible mechanisms for the development of PML in patients with hepatic cirrhosis:

"[H]epatic cirrhosis can lead to portal hypertension and hypersplenism, with subsequent leucopenia as white blood cells are sequestrated in the enlarged spleen. Furthermore, cirrhosis also leads to hypogammaglobulinemia... It is well known that cirrhotic patients have a higher risk of developing bacterial infections, and 30–50% of deaths among cirrhotic patients are directly caused by infections. Immune dysfunction in hepatic disease may be caused by altered cytokine production, impaired cellular immune response and vascular disturbances, which lead together to increased susceptibility to infections."

Neuropathology in Tanzania

I spent half the day today at Muhimbili Hospital in Dar es Salaam, Tanzania discussing interesting neuropathology cases with pathology residents. What a privilege to work with such eager and capable residents. We talked about the 2016 WHO CNS tumor classification update and looked at some challenging cases they have a recently encountered.

Today is Glioblastoma Awareness Day

Last month, a resolution recognizing today, July 17, as Glioblastoma Awareness Day was passed with unanimous consent in the U.S. Senate – a date on which we honor GBM patients, families, and caregivers, as well as recognize the tireless work of researchers and medical providers. Find out more at "Glioblastoma Awareness Day" 

Best Post of March 2019: Glial cytoplasmic inclusions filling putaminal pencillary fibers in a case of multiple system atrophy (striatonigral type)

The next in our "Best of the Month" series is from Thursday, March 14, 2019, which is simply comprised of a nice representative photomicrograph of an MSA-P case:

Putamen (alpha-synuclein immunohistochemistry)

Best Post of February 2019: Dr. Fausto Rodriguez presents on the topic of tumefactive pseudoneoplasms on PathCast

The next in our "Best of the Month" series comes from Thursday, February 28, 2019:

Here's a link to the whole PathCast series on YouTube. Great lectures from prominent pathologists -- completely free! Dr. Rodriguez has another lecture uploaded on the site, and Dr. Arie Perry has two.

Neuropathology Blog Hits Million Pageview Mark!

I just noticed today that this blog now has more than one million pageviews since it inception in October 2007. And this marks the 856th post. Cheers!

Dr. Hilary Nickols: neuropathologist and artist

Hilary Nickols, MD, PhD

From time to time, I feature neuropathologists who exhibit talents beyond the strict confines of neuropathology. For example, I recently features the inimitable Mark Cohen and his prodigious classical guitar skills. I discovered another neuropathologist/artist during the recent annual meeting of the American Association of Neuropathologists:  Hilary Nickols, MD, PhD, of Norton Healthcare in Louisville, Kentucky. Dr. Nickols shared with me her detailed drawing of a craniotomy surgical field which she recently witnessed during a visit to the operating room.

The scene in the operating room, including drains, reflected dura, etc.

Recap of the final day of the AANP meeting

by Dr. Karra Jones:

The final day of the American Association of Neuropathologists annual meeting kicked off with our fearless leader, President Dr. Matthew Frosch.  Dr. Frosch entertained us with thought experiments related to our current methods of sampling tissue and their limitations.  He suggested that newer modalities developed by the following speakers will allow for interrogation of entire volumes of tissue drastically increasing our knowledge about the brain and disease. 

Dr. Kwanghun Chung from MIT talked about rapid 3D imaging and phenotyping of large-scale tissues.  He has developed highly innovative techniques to perform brain-wide molecular phenotyping by engineering the physiochemical properties of tissues, including clinical samples. His 3D image maps of the brain were beautiful and inspiring.   

The AANP Awards Ceremony was next with awards given out for the best platform/poster presentation in multiple categories, the international travel awards, and the Diagnostic Slide Session trainee awards with smiles all around. 

After a short coffee break, Dr. Ed Boyden from MIT talked about the development and use of expansion microscopy to image cells and biomolecules throughout the brain.  His multiplexed imaging techniques create colorful and highly informative images to study the brain and other tissues, and expansion microscopy can even be used on FFPE or fresh frozen tissue. You can find out more about his techniques and even read protocols at ExpansionMicroscopy.org. 

Dr. Jean Augustinack from Massachusetts General Hospital then talked about high-resolution ex vivo neuroimaging with histologic validation.  Her ex vivo images of brains showed amazing resolution and had a nice correlation with histologic sections.  Video of 7 Tesla MRI ex vivo images of a whole human brain at 100 micron resolution were amazing to see and the results were similar to what can be seen with the human eye during brain cutting. 

To finish the 95^th Annual Meeting of the American Association of Neuropathologists, Dr. Frosch installed the new officers, and Dr. Dan Brat, the incoming President, closed the meeting with a ceremonial tap of the gavel. 

It was an amazing and inspiring meeting, as usual.  Thanks to all the program organizers, committee members, our excellent AOE staff, and outgoing executive committee members for all of their hard work over the last year.  Next year in Monterey!