University of Missouri pathologists describe novel glioblastoma cell types

From pathology resident Paul McGowan, MD, the "Sphincter Cell of McGowan":

And from the textbook of University of Missouri's Douglas C. Miller, MD, PhD, a glioblastoma cell which he describes as "an ugly little homunculus giving all us neuropathologists, neurosurgeons, and neurooncologists the finger":

Corythosaurus cell in a glioblastoma

Not to be outdone, the illustrious Dr. Mark Cohen just sent me this corythosaurus cell found in a glioblastoma. An actual corythosaurus is depicted below for comparison:

Starburst mitotic figure in a glioblastoma

Came across this today:

Synoptic reporting for CNS tumors

Mark W. Becher, MD

Neuropathologist Mark W. Becher, MD published an article this past June in Archives of Pathology and Laboratory Medicine (Vol 135:789-792) entitiled Practical Neuropathology Synoptic Reporting for Central Nervous System Tumors. Dr. Becher writes that "the CAP [College of American Pathologists] CNS protocol, published in 2008, is an immense comprehensive document that is not conducive to simple inclusion in a narrative report". Dr. Becher notes that few neuropathologists actually apply the CAP protocol in their daily practice. However, he concludes that because of "the multidisciplinary nature of CNS tumor diagnoses, neuropathologists typically collect clinical, demographic, and imaging data on all CNS tumor cases. These data can readily be entered into a primary synoptic report that could replace our standard narrative report." Dr. Becher describes a synoptic checklist designed to accompany, and possibly replace, the standard narrative surgical neuropathology report, stating that it is more streamlined and easier to use than the exhaustive CAP checklist; as such, it is more useful to both clinicians and data collectors. Here is a link to the CAP surgical neuropathology checklist. The CAP should consider Dr. Becher's proposed checklist and adapt its own accordingly.

Best Post of March 2011 - Dawson Fingers: A Cocktail-Party Term Worth Knowing

The next in our "Best of the Month" series is from March 10, 2011:

Dawson Fingers (in box)

One of the nice things about teaching is that you frequently learn a lot from your students and residents. I had never heard of a radiological finding in multiple sclerosis known as "Dawson Fingers" until I was informed of it by Southern Illinois University second-year medical student Joshua Billington and neurology resident Laxmi Prasad Dhakal. "Dawson Fingers" are a purely radiological finding, which may be why the term is not found in neuropathology textbooks (at least not in the indices of eight different neuropathology textbooks that I consulted). Here's what Adams and Victor's Principles of Neurology (9th edition, page 889) has to say on the subject: "Especially diagnostic are oval or linear regions of demyelination, oriented perpendicularly to the ventricular surface; they correspond to the radially oriented fiber bundles of the white matter and periventricular veins. When viewed in sagittal images, they extend outward from the corpus callosum in a fimbriated pattern and have been termed 'Dawson Fingers'. These areas may extend into the centrum semiovale and may reach the convolutional white matter. Even one highly characteristic lesion is sometimes enough to confirm the diagnosis in the proper clinical circumstances; multiple lesions are more convincing. The presence of such lesions in the corpus callosum is diagnostically useful, as this structure is spared in many other disorders."  See the image above (from Adams and Victor, page 876) depicting Dawson Fingers on sagittal T2-weighted FLAIR MRI. The irony here is that this finding was named after a neuropathologist, James Walker Dawson (1870-1927), despite the fact that many neuropathologists are unfamiliar with the term (well, OK, maybe just me). Thanks to Dr. Dhakal and Pre-Dr. Billington for informing me of this eponymous finding -- one that neuropathologists should probably be able to throw around at any neuroscience cocktail party.

Best Post of February 2011 -- Finally, an alternative to 14-3-3 protein in the diagnosis of CJD!

The next in our "Best of the Month" series is from February 2, 2011:

prion protein

Researchers report in an article in Nature Medicine that a new cerebrospinal fluid test, known as real-time quaking-induced conversion (RT-QUIC) assay, appears to be more specific than the problematic 14-3-3 test, which not infrequently gives false-positive results. ('Quaking-induced' refers to in vitro shaking, which helps to accelerate the reaction.) Not only that: it looks as though RT-QUIC may work on serum samples too, opening up the possibility of much earlier diagnosis and more widespread screening of donated blood. This development could truly revolutionize the pre-mortem diagnosis of prion disease! (Thanks to Dr. Doug Shevlin for calling my attention to this article.)

Genetics of PSP further elucidated

Actor Dudley Moore died in 2002 with PSP

About 20,000 people in the United States are currently diagnosed with Progressive Supranuclear Palsy (PSP). A variant in the MAPT gene, has been repeatedly linked to the disease. Researchers led by Gunter Höglinger of Philipps-Universitat in Germany studied more than 2,000 individuals of European descent diagnosed with PSP, comparing their DNA to that of nearly 7,000 healthy European individuals. The results were published last month in the journal Nature Genetics. The study reported several single nucleotide polymorphisms (SNPs) associated with the disease. One of the SNPs reported by Höglinger’s team is located in MAPT and is closely linked to the already well-established variant for PSP risk. In this study, each copy of the G version of rs8070723 in MAPT was associated with about 5.5 times lower odds of the disease. (Source: spittoon.23andme.com)

Formal Guidelines for Alzheimer's Genetic Testing Issued

Dr. Edward B. Lee

In recent posts, I discussed the availability of direct-to-consumer ApoE testing and the fact that I myself submitted buccal cells to 23andMe.com to discover my own ApoE status. The illustrious Eddie Lee, MD, PhD just emailed me news that the American College of Medical Genetics and the National Society of Genetic Counselors have recently issued formal guidelines regarding ApoE testing in patients. Reported and discussed on the Alzheimer Research Forum, the guidelines, published in the June issue of Genetics in Medicine, identify which individuals may benefit from genetic testing.

Time Magazine On-Line Article: NFL Players May Be More Vulnerable to Alzheimer's Disease

This article, which recently appeared in Time Magazine's on-line Healthland, reports on research recently presented at the Alzheimer's Association International Conference in Paris. Retired NFL players are more likely to develop mild cognitive impairment (MCI) than similarly aged men who didn't play football. 

According to the Greeks, the term "camptocormia" is being misused!

Arkady Melikyan, MD

Dr. Arkady Melikyan, a neurology resident currently rotating on my neuropathology service, informed me today of a word in the neurological lexicon that I had not heard of before: camptocormia. Dr. Melikyan credits attending neurologist Rodger Elble, MD, PhD for enlightening him with regard to this term. Camptocormia, according to Adams and Victor's Principles of Neurology (9th edition, page 117), is "a severe forward bending of the trunk at the waist that is symptomatic of either a dystonia, Parkinson disease, or one of several muscle diseases that focally weaken the extensors of the spine." This condition causes "the patient to walk while looking at the ground beneath the feet, but rarely causes falling". Look at this 17-second video on YouTube demonstrating the condition. Camptocormia is derived from the Greek words campto (meaning to bend forward actively) and cormos (meaning body or trunk). In an article entitled Camptocormia or cormoptosis? The etymology of the word [Ann Rheum Dis. 1996 November; 55(11): 858], Greek authors (and they ought to know!) Drs. Dimitrios Karras, John Vassilakos, and Dimitrios Kassimos have proposed the word cormoptosis instead for this condition (with ptosis meaning passive movement downward). They recommend that the term "camptocormia" be reserved only for patients whose stoop is psychogenic in origin.

Dr. Jean-Paul Vonsattel is on YouTube

Jean-Paul Vonsattell, MD

Dr. Jean-Paul Vonsattel gives viewers a lightening-flash tour through his Brain Bank at Columbia University's Taub Institute for Research on Alzheimer's Disease and the Aging Brain. The video on YouTube covers a lot of ground in 4 minutes and 40 seconds. Very impressive facility!