Monday, December 31, 2007

Doug Miller moves to Missouri

Dr. Douglas C. Miller, former neuropathologist at NYU, has recently moved to the University of Missouri at Columbia. Good luck in Mizzou, Doug!

Thursday, December 27, 2007

Medial lemniscus

Brain cutting sessions with residents can get repetitive after a while unless you have new neuroanatomy information to share with them every so often. To that end, here's a nice description of the course of the medial lemniscus as it ascends through the brainstem written with a visual metaphor that will really stick in your mind. You can use this metaphor in describing the medial lemniscus during your next brain cutting session. The source is my favorite basic neuropathology book: "Practical Review of Neuropathology" by Fuller and Goodman. From page 113:

"The ascending proprioceptive and fine touch system has its relay nuclei, the nucleus gracilus for lower extremity and trunk, and the nucleus cuneatus for upper extremity, neck, and upper the cervicomedullary junction. The second order neurons from these nuclei decussate to form the medial lemniscus that ascends through the brainstem to eventually innervate the ventral posterior lateral (VPL) nuclues of the thalamus. In the medulla, the medial lemniscus is arranged as a vertical (ventral to dorsal ) strip of fibers in the midline with the upper extremity fibers being most dorsal and the lower extremetiy fibers being most ventral; i.e., the sensory homunculus in the medulla is 'standing straight up'. The medial lemniscus rotates to the horizontal in the pons and by the time it reaches the midbrain has rotated further so that the fibers from the lower extremity are now more dorsal than those of the upper extremity. Imagine a reveler on Bourbon Street in New Orleans. Early in the evening, the individual is standing vertically against a lamppost enjoying the libations and this is analogous to the position of the medial lemniscus in the medulla. By mid-evening, the partygoer slides down the lamppost and lays on the street analogous to the increasingly horizonatal disposition of the medial lemniscus in the pons. Finally, the besotted reveler is pulled from the street by his ankles by the local constabulary analogous to the 'feet up' orientation of the medial lemniscus in the midbrain."

I should note that I had to correct a few typos in this as I transcribed it. For example, the authors write "Bourdon Street" instead of "Bourbon Street" in New Orleans. And they refer to the "nucleu gracilus" instead of the "nucleus gracilus". That being said, I still think that Fuller and Goodman provide a wonderful analogy that really helps in explaining the anatomy of the medial lemniscus!

Thursday, December 13, 2007

Neuropathology Course on the Web

Dr. Dimitri P. Agamanolis of Akron Children's Hospital has a neuropathology website that essentially provides a primer on the basics of neuropathology. Check it out at:
I'll be away on vacation until after Christmas, so there will be no new posts until that time. Merry Christmas!

Tuesday, December 11, 2007

Upcoming American Academy of Neurology Annual Meetings

For those of you who like to plan ahead, the 2008 AAN annual meeting will be in Chicago. The 2009 AAN meeting will be in Seattle, Washington. The 2010 Annual Meeting will be in Toronto, Ontario, Canada. There's a link to the AAN website below.

Monday, December 10, 2007

Dr. Asao Hirano

For those wondering who the older gentleman is in the picture with me below, it is none other than the esteemed Dr. Asao Hirano, after whom the actin-associated intraneuronal inclusions known as Hirano bodies are named. He is currently on faculty at Albert Einstein College of Medicine in New York City.

Friday, December 7, 2007

Answer to Quiz Question

Joseph Merrick was originally thought to be suffering from neurofibromatosis type I, a genetic disorder also known as von Recklinghausen's disease. However, it was postulated in 1986 that Merrick actually suffered from Proteus syndrome (a condition which had been identified by Michael Cohen seven years earlier). Unlike neurofibromatosis, Proteus syndrome (named for the shape-shifting god Proteus) affects tissue other than nerves, and is a sporadic rather than familially transmitted disorder. In July 2003, Dr. Charis Eng announced that as a result of DNA tests on samples of Merrick's hair and bone, she had determined that Merrick certainly suffered Proteus syndrome, and may have had neurofibromatosis type I as well. (The above information is adapted from the wikipedia article. But I can vouch for its authenticity.)

The clinical manifestations of Proteus syndrome are, as the name implies, protean. They include:
- Partial gigantism of hands or feet
- Hemihypertrophy
- Subcutaneous lipomas
- Multiple nevi
- Areas of thickened skin and subcutaneous tissue
- Macrocephaly
- Skull anomalies
- Accelerated growth in long bones
- Mentation can be normal or retarded

Thursday, December 6, 2007

Quiz Question

From which of the phakomatoses did Joseph Merrick (played by John Hurt in the movie 'Elephant Man') suffer? The answer will appear in tomorrow's posting.

Wednesday, December 5, 2007

Pediatric Neuro-Onc Meeting this summer

For those looking for a meeting to attend, the International Symposium on Pediatric Neuro-Oncology (ISPNO 2008) wil take place June 30 – July 2, 2008 in Chicago. The abstract submission deadline is January 15th. Here's the link for more info:

Monday, December 3, 2007

14-3-3 protein

A clinician at my hospital ordered a CSF 14-3-3 protein on a lethargic elderly woman with a urinary tract infection. What a waste! The problem with clinicians ordering this test unnecessarily is that it obligates the lab technicians to decontaminate equipment to protect against the possibility of prion contamination. It's an inefficient use of resources for a test that is very non-specific. The patient in question had none of the characteristic clincal signs, nor did she have an EEG done to gathter evidence that she may have CJD. Ordering that test in this patient is just bad medicine.