Monmouth Neuroscience at the 2014 NJ Stroke Conference

We are happy to report that Monmouth’s stoke program was well represented at the 2014 New Jersey Stroke Conference earlier this month.

Two department of medicine residents, Drs Amor and Chan, presented our TIA center data in the poster session:

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Dr Holland gave a talk on the role of telemedicine in stroke rehabilitation.

Gene therapy trial for Duchenne Muscular Dystrophy

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Duchenne and Becker muscular dystrophy are both caused by mutations in the same dystrophin gene.

How it this possible?

Well, the genetic code which is translated to from proteins “talks” in words made of three letters (base pairs).

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A gene mutation that deletes only one or two base pairs, or worse still signals the end of the word (known a “premature stop codon”) will result it a very abnormal dysfunctional gene product, leading to complete deficiency of functioning dystrophin, and the more severe Duchenne Muscular Dystrophy.

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Normal muscle bx (a) vs Duchenne muscular dystrophy (b) with complete absence of dystrophin (d)

However a gene mutation (deletion) that removes base pairs in a multiples of three is called an in-frame mutation, and causes a (sometimes only minor) qualitative change in the dystrophin protein, leading to the milder Becker’s muscular dystrophy.

Ataluren (also known as PTC124) is a small molecule designed to overcome premature stop codons.

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Put simply, the idea is that it might convert some Duchenne boys in to a milder form (more like Becker’s) of muscular dystrophy by allowing them to produce some more normal dystrophin.

The drug can only help boys affected with premature stop codons confirmed by DNA testing.

The drug is currently undergoing Phase III trialsClick here for more information.

Data from our TIA Rapid Evaluation Center (TREC) at the 2014 International Stroke Convention

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Click here to see the abstract.

Click here and here to find out more about this innovative program.

The American Academy of Neurology’s Palatucci Advocacy Leadership Forum

I was lucky enough to participate in the AAN’s 2014 Palatucci Advocacy Leadership Forum last weekend.

The forum provides a wonderful opportunity for neurologists to learn how to:

  • Promote state and federal legislation
  • Work with the media
  • Obtain financial support for research
  • Develop coalitions
  • Organize and reinvigorate state neurological societies
  • Lobby for fair reinbursement
  • Help Draft position statements that affect future legislation

The forum is named in honor of former UCSF Professor of Neurology and AAN Board of Directors Member Donald M. Palatucci, MD.

Attendees get to practice:

  • Creating effective action plans to identify issues and resolves problems
  • Sharpen their interview skills, work with reporters, and improve confidence on camera
  • Get an inside look at how governments work and how to get results

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Here are some examples of on-site activities and the accomplishments of forum alumni:

Surgery for Migraine? Keep your scalp on!

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An excerpt from the Boston Globe April 2012

Debra Haining lay in a hospital bed at Massachusetts General Hospital, awaiting surgery. Both eyelids were colored purple, and blue dots were drawn on her forehead, including one on each temple, and one above her left eye.

The dots indicated the location where she feels the migraine, the trigger points, where the pain strikes. She is 57 years old and says that she never had a headache until five years ago, when she woke up feeling as if she’d been shot through the head.

She was forced to spend nearly every day in bed with the curtains drawn. She could not tolerate light, smell, or sound. Typically she rose only to see her 12-year-old son off to school in the morning and in the afternoon when he returned. Until recently, she had an ice pack to her head and could not drive a car.

A half-dozen medications, four different pain clinics, a variety of headache cocktails and injections, and numerous neurologists didn’t provide relief. Haining, who lives in Pawtucket, R.I., searched the Internet until she found Dr. W.G. (Jay) Austen Jr., of plastic and reconstructive surgery at Massachusetts General Hospital.

Haining says she was tired of doctors who suggested that she learn to accept a lifetime of pain, pills, and shots, and was relieved to find a doctor who offered to treat the cause of the migraine and not just the symptoms. “When you are debilitated and life comes to a halt, you are willing to try what’s out there.’’

In the operating room at Mass. General, Austen began surgery on Haining by making an incision in one of her eyelids in what would appear to be a routine blepharoplasty, a cosmetic surgery known as an “eyelid lift.”

Haining would benefit cosmetically by removal of this globular flat that settles into each eyelid with age. But the point, Austen says, is that this particular procedure provides “easy access” to the critical sensory nerves around her eyes that he believes were causing migraine pain.

This was just one of the three trigger points that Haining identified prior to surgery, and as he operated, Austen would be seeking a structural reason for that pain, a nerve compressed or impinged by surrounding bone or soft tissue.

This surgical approach was developed 12 years ago by Dr. Bahman Guyuron, chairman of the plastic surgery department at University Hospitals Case Medical Center in Cleveland, after several of his plastic surgery patients reported that their migraines improved after a cosmetic procedure known as a forehead lift.

A study published in the journal Plastic and Reconstructive Surgery in 2009 — led by Guyuron and submitted by Case Western Reserve University, the American Migraine Center, and the Center for Headache and Pain, Cleveland Clinic — found that just under 85 percent of patients who underwent the nerve decompression surgery reported at least a 50 percent reduction in migraine, calculating pain, frequency, and duration. Nearly 60 percent (28 of 49 patients) reported a complete elimination of pain. This compared with only 1 of 26 patients who had a sham surgery, in which the surgery was limited to exposure of the nerve but muscle and attachments were left intact. Reported side effects included forehead numbness, temporary hair loss and itching, a slight hollowing of the temple, and small change in eyebrow movement.

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Sound too good to be true?

Dr. Paul Mathew, neurologist at Harvard Medical School and fellow graduate of the 2014 AAN Palatucci Advocacy Leadership Program says yes….

In his recently published review on the subject, Dr Mathew explains that these surgeries are unproven, risky, expensive ($10,000-15,000) and are often not covered by medical insurance.  “Many patients have no or temporary benefits from the surgery and still wind up on long term narcotics”, he says,  and furthermore “These procedures have made their way into mainstream medicine without adequate investigation”.  This is why he has decided to make this subject the focus of his future advocacy efforts.

Click here to read the paper.

Click here to find out more about migraine.

Stem Cells for ALS

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Put simply, some human cells can regenerate:

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Regeneration of a human toe nail

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However, many human cell lines, including central nervous tissue, can not regenerate:

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Human stem cells can go through numerous cycles of cell division while maintaining the undifferentiated state, but can still differentiate into specialized tissues like nerve cells:

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When the appropriate stem cells are injected into injured tissues they will multiply, develop, and repair CNS injuries – at least, that’s the theory:

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So far, human Phase I (safety) trials have shown that stem cells can be injected directly into the spinal cords of ALS patients:


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The Phase II (dose escalation) trials began in September 2013 in Michigan and Atlanta, and aims to recruit 15 patients for 5 different dosing protocols, and will look at efficacy:

Click here to find out more, and watch this space for results.

Applying techniques for peripheral nerve repair to patients with central lesions

Course presented at the 2013 ACRM Meeting

1. Introduction (Backus)

Link to the slides.

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2. Surgical techniques (Elkwood)

Download a pdf of the slides.

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3. EMG Evaluation for PNS and CNS Repairs (Holland)

Watch the slides as a video presentation:

Download a pdf of the slides.

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4. Rehab after tendon transfer for CNS lesion (Bohn)

Download a pdf of the slides