Kuru (You get it from eating brains!)

Eating-human-brains-300x150

Posted by Dr Abhimanyu Kaura, PGYIII (Medicine), Monmouth Medical Center

Kuru disease is one of the five human spongioform encephalopathies caused by prions.  This group also includes Creutzfeldt-Jakob disease (CJD), variant CJD, Gerstmann-Straussler Scheinker syndrome and fatal familial insomnia.

Bovine spongiform encephalopathy also known as “mad cow disease”, is another prion disease that affects cattle, and was responsible for bringing more attention to all of these disorders in the 1990s.

Kuru is confined the Fore tribes of Papua, New Guinea.

kuru2

In the 1950s there were 2100 cases of Kuru in these tribes leading to about 1000 deaths per year.

As per the culture of the Fore tribe, if a person in a family dies his meat is eaten by his family members, especially the wife and children,  as a mark of respect to him.  90% of people affected by the disease were women and children.

kuru3

Between 1996 to 2004 only 11 new cases of Kuru were identified in the region. Currently with the ban of cannibalism in the Farah tribes the disease had become virtually non existent.

Kuru first presents with tremors, then unsteady gait and progresses to leg weakness, ataxia, incoherent speech, sporadic laughter finally.  In later stages, affected patients become demented, bed bound and unable to swallow.

kuru4

Death occurs most commonly from respiratory distress and pneumonia or infection of pressure sores. The disease is fatal within 1-2  years of the onset of symptoms and had no known cure.

Here’s a video introduction

Watch the full story here:

The causative organism is a prion, a mutated protein which replicates itself like a virus, and is spread by eating the neuronal tissue of infected people.

prions

The precise incubation period of the disease is unknown, but can be up to fifty years.

The pathological hallmark is the presence spongioform encephalopathy with of Prion reactive plaques mostly in the cerebellum:
kuru1

The detailed studies of theses cases of Kuru has helped us understand other prion diseases such as variant CJD, sporadic CJD whose clinical features and course is similar to this condition.

And maybe another link between Zombies and neurology?

U4KWZ10GTza2SsuKa7kn_ZombiesCopyrightMikeRollerson

Advertisements

The Neurology of JFK’s Assassination

As a devout conspiracy theorist, I could not resist posting about the John F. Kennedy assassination during its 50th anniversary.

xx

This story contains at least 3 neurological issues of interest.

xx

First, there’s the head wound, and what it tells us about the location of the shooter:
JFK1

Conspiracy theorists have stressed witness testimony that the back of Kennedy’s head was blown out, suggesting a shot from the Grassy Knoll.

Lone assassin theorists have stressed the photographic evidence and the autopsy x-rays, which show the back of the head intact. The panel (inserted above) shows four of the dozens of versions of Kennedy’s head wound.

Click here to find out more.

xx
xx

xx
The Manchurian Candidate 1

Second, even if you believe Lee Harvey Oswald was the lone assassin, some conspiracy theorists would have you believe he was a Manchurian Candidate, manipulated by mind-control experts to carry out the assassination of JFK.

These theories are supported by what is known about the CIA’s experiments with mind control in the 1950s, and Oswald’s alleged CIA connections.

Click here to read more about this.

xx

xx

Finally, there is Jack Ruby‘s murder of Lee Harvey Oswald:

The matter of neurologic interest here is that Ruby’s defense at trial was based on an abnormal electroencephalograph.

Defense expert Frederick Gibbs, one of the pioneers in the use of electroencephalography for the diagnosis and management of epilepsy,  testified that Ruby’s EEG showed right temporal 6/s sharp waves, and that this was evidence of “psychomotor epilepsy”.

14-6

Gibbs suggested that affected patients manifest personality instability, lack of emotional control, convulsive and excessive types of behavior.  He, and other physician experts at the trial further postulated that Ruby killed Oswald during a fugue state induced by a psychomotor seizure.

Middle finger

This image of Ruby shooting Oswald was used at trial – Ruby is seen using his middle finger to pull the trigger, with his left hand thrown out in the opposite direction, supposedly indicating a seizure.

The prosecution’s neurology expert disagreed, stating that the EEG findings were a “slight abnormality” and didn’t indicate epilepsy.  Furthermore, he indicated that Ruby’s demeanor and behavior, as described by witnesses, were not consistent with a psychomotor seizure.

Ruby was convicted, and sentenced to death.

Click here to find out more about the neurology at the trial.

This EEG “finding” is now known to be a normal variant with no clinical significance.

This case underscores the importance of treating the patient, not the test result, an adage well known to true clinicians everywhere.

Stem Cells for ALS

stem1

xx

Put simply, some human cells can regenerate:

stem3

Regeneration of a human toe nail

xx

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

stem2

xx

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:

stem4

xx

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

stem5

xx

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


xx

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.

Lawrence of Arabia and the Motorcycle Helmet

lawrence

Thomas Edward Lawrence,  (1888 – 1935) was a British army intelligence officer during the First World War.

His efforts in instigating the Arab Revolt against the Ottoman Turkish (allies of Germany) were featured in a documentary by American journalist Lowell Thomas, ultimately earning him international fame as Lawrence of Arabia.

lawrenceOfArabia_1484445c

Lawrence had trouble adjusting back into civilian life after his retirement from military service, exacerbated by his fame and pursuit by the media.

He built a small hut in a then rural area of my home town, Chingford, where he completed his book “Seven Pillars of Wisdom”.  This is commemorated by a plaque fixed on the obelisk on Pole Hill.

obolisk

Lawrence was an avid motorcyclist and owned seven different Brough Superiors, dubbed the “Rolls-Royces of Motorcycles.”

On the morning of May 13, 1935, Lawrence was speeding down a narrow county road on his motorbike, when he suddenly swerved to avoid hitting two boys on bicycles and was thrown forward over the handlebars.

5287915-large

At the time, helmets were only worn during races, and he sustained a skull fracture and massive head injuries.

He was taken to Bovington Camp Military Hospital in a coma, where the best specialists in the country were rushed to save him, one of them the young neurosurgeon Hugh Cairns.

Lawrence died 5 days later, without regaining consciousness.

Cairns was so profoundly moved by the tragedy of this famous First World War hero’s tragic death from severe head trauma, that he devoted his career to head trauma in motorcyclists.

During the Second World War, Cairns noted the high death rate amongst army dispatch riders, even before the actual start of hostilities, exacerbated by restricted radio communications and blackout regulations.

He observed that 2279 motorcyclists and pillion passengers were killed in road accidents during the first 21 months of the war.  However, there were only 7 cases of motorcyclists injured while wearing a crash helmet, none fatal.

His collected the monthly totals of motorcyclist fatalities in the United Kingdom from 1939 to 1945:

graph

And he noted that the decline in the number of fatalities took place after November 1941, when crash helmets became compulsory for army motorcyclists on duty.

Further work included an analysis of the pathophysiology and mechanisms of head injury, which led to the development of new more protective designs and materials for crash helmets:

helmet

Click here to find out more about Lawrence, Cairns and crash helmets.

Left world neglected

Lhvf

Posted by Sanya Naware MSIV, Drexel University College of Medicine

What would it feel like to only perceive one half of the world around you?

For patients with hemispatial neglect, this is an everyday reality.  Hemispatial neglect or hemineglect is a condition in which damage to one hemisphere of the brain causes a lack of awareness of one contralateral side of space.  It is most often a lesion of the right posterior parietal cortex affecting the contralateral side of the body.  The person is unable to recognize stimuli or process them on the affected side.  Left neglect is more common than right neglect because the right hemisphere is able to compensate for the loss of left hemispheric function.

Because these patients only perceive one side, they only draw what we know to be half of an image as seen in the video and image below:

naware

Lisa Genova, a neuroscientist, expertly describes the daily challenges of living with neglect in her book Left Neglected. It is a difficult condition to imagine and this book does a wonderful job of explaining the realities and frustrations of the patient and her family.

naware2

The main character, Sarah Nickerson, suffers a traumatic brain injury in a car crash.  When she wakes up, everyone around her realizes that she ignores the left side of everything.  Whether it is a clock, a painting, or a room around her, she is not able to recognize the left side of anything.  While she is able to feel the left side of her body, she has to focus on the fact that she has a left side in order to control her left leg and walk.  In fact, when she first sees her left arm, she states that it feels like it belongs to another person, a problem called somatoparaphrenia.  While eating, she only eats the food on the right side of her tray.  She frequently bumps into objects on the left side of her body because she is unaware of their presence.

Sarah’s story is optimistic as her therapist and family use certain tricks to help her adjust.  Some of these methods include placing bright orange tape on the left side of things around their home, using a ruler to guide her to the left side of the page, and wearing shiny jewelry on her left hand to attract attention to it.

Genova ends her book by endorsing the New England Handicapped Sports Association (NEHSA), an organization of volunteers who help people like Sarah find some independence and confidence.

Click here to find out more about right hemispheric brain damage from NEHSA.

References

  1. Genova, Lisa. Left Neglected: A Novel. New York: Gallery, 2011.
  2. Waxman SG. Chapter 21. Higher Cortical Functions. In: Waxman SG, ed.Clinical Neuroanatomy. 26th ed. New York: McGraw-Hill; 2010.

Petadolex for Migraine Prophylaxis: The Facts

index

Posted by Sonia Jasuja, MSIV Drexel University College of Medicine

As a current student of traditional Western Medicine, I have been trained to turn towards modern pharmacology and away from natural remedies, for the most part.  However, as someone who has suffered from severe skin allergies all my life, I know how desperate patients can get in order to find something that really works! When Western Medicine fails us, where can we turn?

During my clinical Neurology rotation at Monmouth Medical Center, I saw another issue that plagues patients: Migraine.  While there have been great advances in the prevention and treatment of migraine, some patients are still left with debilitating pain. It was during this rotation that I first heard about Petadolex, or Petasites hybridus (aka Butterbur).

What is it? Butterber is an herbal plant that has been used for medicinal purposes, including migraine and headache, allergies, asthma, and many more. Most herbal remedies use the root extract in the form of a pill. It has properties that relieve spasms and decrease inflammation.1

Is it safe? Yes- studies have determined that Petasites is safe to use for the prophylaxis of migraine. The dose that was cited to have moderate efficacy is 150mg daily.2

Side effects are very mild and include burping, stomach upset, diarrhea, fatigue and itching.3

One important thing to keep in mind- make sure to only buy Petasites hybridus that is certified and labeled, “PA free”. “PA” stands for pyrrolizidine alkaloids, which cause adverse effects in the liver, lungs and circulatory system. PA’s can cause cancer.3

You should not take Petasites if you are pregnant or breast-feeding, have liver disease, or if you are allergic to ragweed, marigolds, daisies or other related herbs. 3

Does it work? Probably, but we still need more information! In 2006, Agosti et al. published “Effectiveness of Petasites hybridus preparations in the prophylaxis of migraine: A systematic review”. Of the two studies that were looked at, the systematic review showed that there is only moderate evidence for the effectiveness of Petasites at the dose of 150mg/day for a period of 3-4 months. The review also thoughtfully pointed out that confounding factors still need to be accounted for. These factors would include things like which migraine treatments have been successful or unsuccessful in the past, and any use of addictive or hormonal substances, such as nicotine or estrogens.2

The review article states that the overall effect size of the 150mg extract dose is approximately 15% percent lower migraine frequency rate per month compared to placebo.2

The bottom line.  If you have frequent or debilitating headaches, you should see you doctor for an evaluation.  You may need some diagnostic testing, and there are probably some very effective conventional medications you can try.  However, if you are still having frequent headaches despite that, Petasites might be worth a try.

REFERENCES

1. Brind’Amour, Katie. “Migraine Herbal Home Remedies From Around the World.”Healthlines RSS News. Healtline Editorial Team, 16 Apr. 2013. Web. 16 Nov. 2013.

2. R. Agosti, R.K. Duke, J.E. Chrubasik, S. Chrubasik, Effectiveness of Petasites hybridus preparations in the prophylaxis of migraine: A systematic review, Phytomedicine, Volume 13, Issues 9–10, 24 November 2006, Pages 743-746, ISSN 0944-7113, http://dx.doi.org/10.1016/j.phymed.2006.02.008.

3. “Butterbur Information | Evidenced-Based Supplement Guide.” MedicineNet. MedicineNet.com, n.d. Web. 16 Nov. 2013.

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.

xx

2. Surgical techniques (Elkwood)

Download a pdf of the slides.

xx

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

Watch the slides as a video presentation:

Download a pdf of the slides.

xx

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

Download a pdf of the slides