XVIIth International Congress of Neuropathology

Save the date: September 11 - 15, 2010, Salzburg, Austria.
Here's the link: http://www.icn2010.org/
I'll also put it in my "links of interest" below for future reference.

Best Post of November '07

Today I feature an edition of my occasional “Best of…” series in which I reread the blog posts of each month and repost my favorite one. Here’s my favorite for November ’07:

“The pineal gland is innervated by the nervi conarii. This name was derived from the fact that an antiquated term for the pineal gland is the "conarium", which literally means "cone". The nervi conarii are sympathetic fibers with cell bodies situated in the superior cervical ganglion.”

I might add that sympathetic postganglionic nerve fibers from the superior cervical ganglia provide norandrenergic input that induces melatonin production by the pineal gland.

Nice Website for Neurology Residents

The esteemed Dr. Mark Cohen of Case Western Reserve University alterted me to a good website where neurology residents can communicate with one another about anything neurological. Check it out:
http://frontalcortex.com/?page=home

I'll put it in my "links of interest" below for future reference.

Wednesday: My last day at the AAN annual meeting

I'm currently at an evening conference entitled: "Case Studies in Dementia". This will be my last engagement at the meeting. My family and I leave tomorrow morning for a 3+ hour train ride back to Springfield, IL. Tonight's program features: Dr. Richard Caselli of the Mayo Clinic Scottsdale; Dr. Jody Corey-Bloom from UCSD; and Dr. Ronald Petersen of the Mayo Clinic Rochester. Cory-Bloom started off with a case of a 33 yo female with a one year history of cognitive difficulty. Her boyfriend described reduced speech alternating at other times with incessant speech. The Boston Naming Test showed difficulty with word finding. There was no deficit in memory. Brain MRI showed mild frontal atrophy. Two years later, the patient is now almost mute, with echolalia. She is very oral, and very disinhibited, and paces incessantly. She is also compulsive about routines. Dr. Corey-Bloom feels that the patient has frontotemporal lobar degeneration (the behavioral variant). The other two types of frontotemporal lobar degeneration, as described by Neary, are progressive nonfluent aphasia and semantic dementia. (Semantic dementia is where the patient has fluent speech with loss of meaning. They can also have mental rigidity and a cold affect.) Corey-Bloom went on to talk about FTDP-17, and the recent discovery of the two genetic subtypes: MAPT mutation and Progranulin mutation. Corey-Bloom says that the only treatment for FTLD is aimed at the behavioral symptoms (SSRIs and anti-psychotics).

Dr. Caselli presented a case of a 46 yo woman with a five-month personality change with increased libido, increased wine consumption, chain smoking, erratic sleep patterns, and a fixation on consuming candy mints. She had five seizures witnessed by her physician husband. MRI showed no frontal atrophy and was essentially normal. I raised my hand when there was a call for differential diagnosis, I suggested a low-grade brain tumor. Other people suggested various encephalitides. Brain biopsy showed Nonvasculitic Autoimmune Inflammatory Meningoencephalitis (Caselli doesn't like the term Hashimoto's Encephalitis since these patients don't have serum anti-thyroid antibodies). What was unusual here was that the diffuse process caused a focal (frontal) clinical presentation.

Dr. Petersen presented a case of a patient with a clinical picture of FTLD who, upon imaging for amyloid using PET scanning for Pittsburgh compound B (PiB) caused Petersen to decide that the diagnosis was more consistent with Alzheimer disease.

Tuesday at the AAN meeting

The day started early with a 5 AM hotel wake up call. The 5K Run for Research took place at 6:30 this morning, with about 100 participants -- including myself. 

I hit the exhibits today for the first time. Of course, all the big pharmaceutical companies were present with their flashy displays and gifts (I picked up a book reading light, a solar-powered recharger, and, of course, pens). But the highlight of the exhibits was what I call "Pauper Alley". This was the area where the not-for-profit foundations looking to gain attention for their particular cause were allowed to set up booths. I had the pleasure of meeting the staff of the Jain Foundation (link below in "links of interest to neuropathologists"), which is dedicated to research into cures for limb girdle muscular dystrophy 2B (dysferlinopathy). I was involved several months ago in the diagnosis of a patient with this rare disease, and I had the honor of meeting a young man with dysferlinopathy (also known as Myoshi myopathy).  At another booth, I met an older gentleman with adult polyglucosan body disease. Both of these people were wheelchair-bound, but completely intact cognitively and dedicated to their cause. I was impressed with these little organizations that were trying to make a difference.

Monday at the AAN Meeting

I'm now awaiting the commencement of the colloquium on neurology education, which I thought might be of use since I teach neurology and neuropathology to medical students and all neurology residents at SIU rotate with me at some point. Last night I had a pleasant dinner with Dr. J. Clay Goodman, where I encouraged him to publish a second edition, with Dr. Greg Fuller, of the best introductory neuropathology guide on the market: Practical Review of Neuropathology (2001). I mentioned that all he needs is a little updating and a switch to color pictures, and he has a best-seller. He replied that he and Greg have talked about putting out a new edition, perhaps a web-based version that will avoid a publisher altogether. Also at dinner with Goodman was Dr. P. James B. Dyck of the Mayo Clinic. The occasion was a dinner seminar that Goodman and Dyck were presenting entitled "You Want to Biopsy My What?".  This outstanding presentation focused on brain biopsy for rapidly progressive CNS disease (Goodman) and peripheral nerve (Dyck). Goodman closed his presentation with the following line: "When you hear hoofbeats, think horses rather than zebras... unless you're in the Serengeti. And in neurology, you always have one foot in the Serengeti!"

Dr. Robert Darroff, of Case Western, is currently giving an amusing talk entitled "Advice to Educators". He says that students are more receptive to learning when they are feeling relaxed. Humor is not to be underestimated in teaching!

Sunday at the American Academy of Neurology Meeting

Thousands of people are in attendance here in Chicago. The exhibit hall will not be opening until tomorrow, so today I am attending seminars. This morning I attended a breakfast meeting featuring the utility of FDG-PET imaging in the differential diagnosis of Alzheimer Disease versus Frontotemporal Dementia. Since different treatments are appropriate for each disease type, I discovered that PET imaging is covered by Medicare under appropriate circumstances. More tomorrow....

Neuropathologists meet last week in San Diego

The esteemed and illustrious Dr. John Donahue attended the recent American Association of Neuropathologists annual meeting in San Diego. He was kind enough to provide a summary of the famous Diagnostic Slide Session from that meeting as follows:

“It came time for the Diagnostic Slide Session, now being moderated by Anthony Yachnis. It can be summed up as follows: "Dr. Mark Cohen, ROCK STAR!!!" He had to get at least 7/10 cases absolutely correct. He was diagnosing things I had never even heard of. There was a case of "vanishing white matter disease," also called "childhood ataxia with central hypomyelination" and "ovarian leukodystrophy," due to a mutation in eukaryotic initiation factor 2B, and he nailed it! I have never even remotely heard of that disease. There was also a case of intractable epilepsy due to "filamin A" (on chromosome X) astrocytic inclusions that look superficially like Rosenthal fibers, and he nailed it! I never heard of that, either. It was one of the most impressive diagnostic exhibitions I have ever seen. I asked him how long he's been doing this; he said 15 years or so. The cases were so hard that I only felt comfortable going up to the mic once, and after I gave my differential diagnosis, the speaker said "What did you do with the unstained slide that I provided?" I said, "I didn't want to waste it, so I was waiting to see the right answer." That generated laughter and applause!”

Thanks for your contribution, Dr. Donahue. And congratulations are in order. It turns out that an image from Dr. Donahue’s article Apolipoprotein E, Amyloid-[beta], and Blood-Brain Barrier Permeability in Alzheimer Disease appears on the cover of the current issue of the Journal of Neuropathology and Experimental Neurology.

Next week, I’ll be posting from the annual meeting of the American Academy of Neurology in Chicago, IL. The meeting celebrates the 60th anniversary of the organization. The first annual meeting, featuring 38 presented papers, was held at the French Lick Springs Hotel in 1949. Two thousand abstracts have been accepted for this year’s meeting.

Best Post of October '07

Back when I started this blog in October of 2007, I had no readers. Now, after about six months, I have almost no readers. So, for those of you who were not with me from the beginning, I will be featuring occasional "Best of ..." posts for each of the months that this blog has been on-line.

Here's an abbreviated version of the best post of October '07:

Frozen/Permanent discrepency article in Archives of Pathology.
This article in the October '07 issue of Archives of Pathology and Laboratory Medicine, by Plesec and Prayson at the Cleveland Clinic, looks at 57 instances of CNS tumor frozen sections (out of a pool of 2156 cases) where a diagnostic discrepancy was found between frozen and later permanent sections. Of those 57, the authors were able to identify a few pitfalls that one should be aware of when attempting to make a diagnosis at frozen section.

Important tips to be gleaned from this article include: 1. Perivascular hemosiderin deposition is more likely to be seen in schwannomas as opposed to meningiomas; 2. Consider CNS lymphoma when contemplating a frozen section diagnosis of small cell glioblastoma; 3. Consider sarcoma when contemplating a frozen section diagnosis of schwannoma.

Finally, to quote the authors: "The discrepencies did not significantly impact patient management in any of the cases because postoperative management was predicated on the final diagnosis." That goes to show you how important our frozen section diagnoses are!!!

First report of a primary intracerebral angiomatoid fibrous histiocytoma

The esteemed Dr. Chad DeFrain provided me with a case report from the American Journal of Surgical Pathology (2008;32:478–484) which describes the first reported case of a primary intracerebral angiomatoid fibrous histiocytoma. The relatively large tumor was located in the left occipital lobe. A team led by Dr. Arie Perry at Washington University in St. Louis describes the tumor as having a unique clear cell sarcoma-like gene fusion transcript, t(12;22). The authors conclude: "Although AFHs are usually associated with a favorable prognosis, the impact of this tumor’s atypical cerebral location and unique molecular features are unclear."

Cord Blood Improves Alzheimer’s Pathology in Mouse Model, Study Finds

The illustrious Dr. Doug Shevlin forwarded to me an article he found in America’s Blood Centers Newsletter which describes a study using a mouse model published online last month in the journal Stem Cells and Development. The study suggests that targeted immune suppression therapy using human umbilical cord blood cells may improve the pathology associated with Alzheimer’s disease. Researchers found that the amount of amyloid plaques – hallmarks of Alzheimer’s pathology in the brain – was reduced by 62 percent after venous infusion of umbilical cord blood. The study suggests that umbilical cord blood therapy may target the pathogenic inflammatory response that contributes to Alzheimer’s disease and other degenerative conditions in humans.

Citation: WV Nikolic, et al. Peripherally administered human umbilical cord blood cells reduce parenchymal and vascular amyloid deposits in Alzheimer mice. Stem Cells Dev. 2008 [Epub ahead of print]

Thanks for the article, Dr. Shevlin!