A case of Alexander disease

Alexander disease is a progressive and often fatal leukodystrophy wherein innumerable Rosenthal fibers form. Caused by mutation in the gene encoding GFAP, Alexander disease can be inherited in an autosomal dominant manner, but typically arises de novo.

Cerebrum

Cerebrum

Cerebellum

New Channel on YouTube: Adventures in Neuropathology

There's a new channel on YouTube called Adventures in Neuropathology. The intended audience appears to be medical students or beginning residents. The channel offers brief reviews of individual topics in bite-size chunks. It looks as though the channel just got underway, with fifteen short videos having been posted with the past two months.

A gaggle of Rosenthal fibers forming at the pushing border of an ependymoma

Ependymoma (left) pushing on adjacent brain induces numerous Rosenthal fibers,
particularly around a blood vessel

Tumours of the CNS Dataset is now published to the ICCR website

The International Collaboration on Cancer Reporting has published the Tumours of the CNS Dataset.

According to the published document, the "dataset has been developed for the histological assessment of benign and malignant tumours of the central nervous system (CNS) and its coverings, as well as tumours from those aspects of the peripheral nervous system immediately adjacent to the CNS. This dataset applies to both biopsy and resection specimens. Haematological lesions that may originate in the brain are included. Tumours of the anterior pituitary gland are included as the majority of these tumours are reported by neuropathologists worldwide (a separate dataset specifically for pituitary tumours may be considered when the 5th series of the World Health Organisation (WHO) Classification of Tumours is being developed). It is intended that this dataset should be used in conjunction with the ‘Molecular information for CNS specimens’ and the ‘Final integrated report/diagnosis for CNS specimens’ datasets. A full diagnosis of CNS tumours should ideally conform to the final integrated diagnoses in the 2016 World Health Organisation (WHO) Classification of Tumours of the CNS (2016 CNS WHO), which requires integration of elements from histological and ancillary analyses. Nonetheless, it is realized that some diagnoses may not fit precisely within existing diagnostic categories."

2019 Annual Meeting of AANP: Save the Date!

Salzmann nodular degeneration of the cornea

Histopathology of Salzmann nodules (*). Disruption of the Bowman membrane is demarcated in each photomicrograph by the area between the left and right arrows: (A) hematoxylin and eosin stain; (B) periodic acid-Schiff stain; (C) immunohistochemistry with antibody against pan-cytokeratin; and (D) immunohistochemistry with antibody against vimentin. (From Stone DU, et al. Histopathology of Saltzmann Nodular Corneal Degeneration. Cornea. Volume 27(2), February 2008, pp 148-151)

Salzmann nodular degeneration  was once called  Salzmann nodular dystrophy. It is now recognized that this typically unilateral disorder is not heritable and is best classifid as a secondary degenerative process of uncertain cause. Clinically, the corneal epithelium is focally elevated by white mounds of dense collagenous connective tissue. Salzmann nodular degeneration resembles a massive focal pannus histopathologically. Mounds of relatively acellular hyaline connective tissue elevate the corneal epithelium anterior to the plane of Bowman membrane, which may be destroyed.

Source: Reference: Eagle, Ralph C. Eye Pathology: An Atlas and Text [2nd edition] page 86.

Best Post of June 2018: Who was Rosenthal?

Of the ten posts from June 2018, my favorite was "Who was Rosenthal?" from June 22. So, here's the next in our "Best of the Month" series:

A Rosenthal fiber in pilocytic astrocytoma

"In 1898, the German pathologist Werner Rosenthal noted elongated inclusions within the gliotic edge of a syringeal cavity of an ependymoma. Assigned to write the case report by a senior mentor while serving as a “first assistant” at the University of Erlangen, Rosenthal colorfully described these inclusions as a “glossy formation of little bulbs or wavy sausages with one thick and one pointed end.”….  His supposition that they were related to glial fibers would prove surprisingly insightful. Not until some 20 years later did Bielschowsky and Unger use the term 'Rosenthal fibers' when describing structures in the gliotic capsule of a cystic teratoid tumor."

Quoted from:  F.J. Wippold, A. Perry and J. Lennerz. Neuropathology for the Neuroradiologist: Rosenthal Fibers. American Journal of Neuroradiology May 2006, 27 (5) 958-961.

Omalu puts a religious spin on his assessment of the football: "It is not of God."

“We shouldn’t let children play [football] because we are damaging their brains and robbing them of their humanity. That is a fact.” That's what Dr. Bennet Omalu had to say to a reporter associate with Sojourners, a faith-based organization. In the recently released article from Sojourners, Omalu said that football is a sport "not of God". A committed Catholic, Omalu  says that he lets "the Spirit of God percolate into my being... Everything I do, I do through the eyes of faith.”

Bennet Omalu, MD

Omalu is best known for the startling discovery he made after performing an autopsy on former NFL player Mike Webster. As chronicled in the 2015 film Concussion, with Will Smith starring as Omalu, the then-medical examiner in Pittsburgh found Webster had chronic traumatic encephalopathy and related it to Webster's years of playing football.

CAP Neuropathology Committee meets in Montreal

The College of American Pathologists Neuropathology Committee met this past weekend in Montreal. It marked the end of my six-year term as a member of the committee. It was an honor to have served with such a wonderful collection of neuropathologists over the years. I'll miss working with these great colleagues.

In Montreal, committee members (left to right) Kara Jones, Arie Perry, myself, Eyas Hattab, Andrea Wiens, and junior member Lindsey Lowder

At dinner after a day of committee work (left to iight): CAP staffer Annabel Dizon, Karra Jones, Eyas Hattab, Andrea Weins, myself, Arie Perry, Lindsey Lowder, and Lindsey's husband Jon Lowder

Lewy Body Pathology and Chronic Traumatic Encephalopathy Associated With Contact Sports

Dr. Ann McKee's group last week published an article in the Journal of Neuropathology and Experimental Neurology supporting the theory that contact sports increases the risk of developing neocortical Lewy body disease, which may in part explain the extrapyramidal motor symptoms sometimes observed in patients with chronic traumatic encephalopathy. Dr. Thor Stein is the senior author on the paper, entitled Lewy Body Pathology and Chronic Traumatic Encephalopathy Asssociated with Contact Sports.