“… within months, hundreds of the young male inmates of the camp began limping, and had begun to use sticks as crutches to propel themselves about. In some cases inmates had been rapidly reduced to crawling on their backsides to make their ways through the compound …. Once the inmates had ingested enough of the culprit plant, it was as if a silent fire had been lit within their bodies. There was no turning back from this fire—once kindled, it would burn until the person … would ultimately be crippled …. The more they’d eaten, the worse the consequences—but in any case, once the effects had begun, there was simply no way to reverse them …. ” -insights from Dr. Arthur Kessler, prisoner and doctor within the concentration camp What would you do if you were trapped out in the wilderness and your glutes and legs stopped working ? This is just what happened to Christopher McCandless (aka, Alexander Supertramp) in the wilderness outside Fairbanks Alaska. Neurolathyrism is a toxic myelopathy caused from ingestion of Lathyrus sativus grass pea. It causes paralysis, lack of strength or inability to move the lower limbs and may involve the pyramidal tracts of the spine/CNS producing UMN signs (upper motor neuron signs).  A unique symptoms of lathyrism is apparently the atrophy of the gluteal muscles.    Dear Gait Guys, why are you telling us obscure things about a toxic neuropathy ?   Ok, let us back up. But for you to understand we first need to tell you about a 1940’s Holocaust concentration camp in the Ukraine. Vapniarka is a presently a small town of ~8600 in Vinnytsia, Obllast, Ukraine.  It was during the months of October 1941 through March of 1944 it became the site for the German occupied Holocaust concentration camp imprisoning Romanian Jews.  As most concentration camps go, food, water, sanitation and disease outbreaks were common problems and concerns. Food was so limited that the prisoners were at one point fed barley bread that had 20% straw mix within and a species of pea known as Lathyrus sativus typically used to feed livestock.  It was only a short time later that strange symptoms began to break out amongst the prisoners. At first they became weak but it wasn’t long before they had difficulties ambulating and then became paralyzed with what was diagnosed as Neurolathyrism also known more simply as Lathyrism, a form of spastic paralysis. The culprit was oxalyldiaminopropionic acid (ODAP) from the peas. Some sources say that by 1943 hundreds of prisoners were struck down with Lathyrism and apparently 117 Jews were left permanently paralyzed. We believe we first read about this in early 2013 in an online news article by Ronald Hamilton in a paper entitled “The Silent Fire: ODAP and the Death of Christopher McCandless”. McCandless has been made famous for his story and death deep in the Alaska wilderness in the Jon Krakauer book “Into the Wild”. We were excited to see this paper quoted in Jon Krakauer’s The New Yorker article this month. In his paper Hamilton wrote,   "… within months, hundreds of the young male inmates of the camp began limping, and had begun to use sticks as crutches to propel themselves about. In some cases inmates had been rapidly reduced to crawling on their backsides to make their ways through the compound …. Once the inmates had ingested enough of the culprit plant, it was as if a silent fire had been lit within their bodies. There was no turning back from this fire—once kindled, it would burn until the person who had eaten the grasspea would ultimately be crippled …. The more they’d eaten, the worse the consequences—but in any case, once the effects had begun, there was simply no way to reverse them …. “ In Krakauer’s recent The New Yorker article "How Chris McCandless Died”, he gives an explanatory full-circle synopsis regarding the gripping conclusion in his book “Into the Wild”.  In the article he speaks about Hamilton’s paper, McCandless’s fate and also mentions that in the 20th century more than a hundred thousand other people worldwide have been permanently paralyzed from eating grass pea containing the neurotoxin ODAP. According to Hamilton, the neurotoxin over-stimulates the nerve receptors causing them to burn out. As he explained in his “The Silent Fire” paper, “It isn’t clear why, but the most vulnerable neurons to this catastrophic breakdown are the ones that regulate leg movement…. And when sufficient neurons die, paralysis sets in…. [The condition] never gets better; it always gets worse. The signals get weaker and weaker until they simply cease altogether. The victim experiences “much trouble just to stand up.” Many become rapidly too weak to walk. The only thing left for them to do at that point is to crawl….” You can read the Krakauer / Hamilton account and recent story in the article link found below. In it they both tell the most recent events in the laboratory testing of the seeds that McCandless ate in bulk during his last months/weeks.  The tested seeds were found to be in high enough concentration to cause the symptoms (of Lathyrism) McCandless wrote about in his last weeks of survival when he was more susceptible to the neurotoxin having already been in severe malnutrition and hunger. THE NEW YORKER LINK : http://www.newyorker.com/online/blogs/books/2013/09/how-chris-mccandless-died.html?mbid=social_retweet&mobify=0 We dove a little deeper into this toxic myelopathy and discovered some helpful journal articles. According to Misra et al.  "patients complained of walking difficulty due to weakness and leg stiffness. The gait abnormalities ranged from spastic gait, toe walking and the need of assisted gait devises such as canes. They spoke of the weakness being mild to moderate and less prominent than was spasticity. In 8 of their subjects the physical signs were asymmetrical. Peripheral neuropathy was present in only one patient, but muscle atrophy and widespread fasciculation's were not found. A higher frequency of peripheral neuropathy and lower motor neuron involvement has been reported from Bangladesh and Israel. Severe spasticity in the absence of prominent weakness in lathyrism may be due to the involvement of certain specific groups of corticospinal fibres.“ We are big fans of Krakauer’s writing (and now Hamilton’s paper "The Silent Fire”) and this was a good story to close the Alexander Supertramp chapter hopefully once and for all. “Into the Wild” by Krakauer was a riveting book, one of our favorites.  Sean Penn’s cinematic interpretation of the story was good as well.  But for two gait geeks like us, to finally find out that poor Chris died of something so rare and complex, something that could be traced back as far as the holocaust concentration camps where people’s gait was first seen impaired was pretty fascinating. We are sure there billions of folks who have never looked at our blog and who will never likely read it.  Gait and gait related disorders and diseases do not get many people excited, but for us, this might as well be pornography. We are sure there are millions that are not fascinated by the fact that every organism on this planet locomotes in one manner or another, each with their own characteristic rules and biomechanics. It is sad to us that few really pay attention to how humans locomote and ambulate, at least not until they break a leg, sprain an ankle or come down with some complex motorneuron lesion. Gait is everywhere once you start looking for it. Whether you start to become aware of it at the local shopping mall, in TV commercials, during Sunday night football, during the Olympics, at your grandmama’s house, during your son or daughters walk to the dinner table , or even in the movies… . . gait is everywhere. Sean Penn’s interpretation of Krakauer’s “Into the Wild” is a touching sweeping movie and for us to now find out that the thing that ultimately led to McCandless’s demise was a gait related neurotoxic myelopathic disorder just goes to prove our point, gait is everywhere.  Why more people do not see this is beyond us, but hey, it would be a strange world if everyone was as nerdy as the two of us wouldn’t it !?  Shawn and Ivo, The Gait Guys.     References:   1. Paraplegia. 1993 Apr;31(4):249-54. Clinical aspects of neurolathyrism in Unnao, India. Misra UK, Sharma VP, Singh VP. http://www.ncbi.nlm.nih.gov/pubmed/8493040 2. J Neurol. 2012 Jul;259(7):1263-8. doi: 10.1007/s00415-011-6306-4. Epub 2011 Nov 12. Neurolathyrism: two Ethiopian case reports and review of the literature. Woldeamanuel YW, Hassan A, Zenebe G. http://www.ncbi.nlm.nih.gov/pubmed/22081101 3. Neurobehav Toxicol Teratol. 1983 Nov-Dec;5(6):625-9. Lathyrism: a neurotoxic disease. Spencer PS, Schaumburg HH. http://www.ncbi.nlm.nih.gov/pubmed/6422318

 “… within months, hundreds of the young male inmates of the camp began limping, and had begun to use sticks as crutches to propel themselves about. In some cases inmates had been rapidly reduced to crawling on their backsides to make their ways through the compound …. Once the inmates had ingested enough of the culprit plant, it was as if a silent fire had been lit within their bodies. There was no turning back from this fire—once kindled, it would burn until the person … would ultimately be crippled …. The more they’d eaten, the worse the consequences—but in any case, once the effects had begun, there was simply no way to reverse them …. ” -insights from Dr. Arthur Kessler, prisoner and doctor within the concentration camp

What would you do if you were trapped out in the wilderness and your glutes and legs stopped working ? This is just what happened to Christopher McCandless (aka, Alexander Supertramp) in the wilderness outside Fairbanks Alaska.

Neurolathyrism is a toxic myelopathy caused from ingestion of Lathyrus sativus grass pea. It causes paralysis, lack of strength or inability to move the lower limbs and may involve the pyramidal tracts of the spine/CNS producing UMN signs (upper motor neuron signs).  A unique symptoms of lathyrism is apparently the atrophy of the gluteal muscles. 

 

Dear Gait Guys, why are you telling us obscure things about a toxic neuropathy ?

 

Ok, let us back up. But for you to understand we first need to tell you about a 1940’s Holocaust concentration camp in the Ukraine.

Vapniarka is a presently a small town of ~8600 in Vinnytsia, Obllast, Ukraine.  It was during the months of October 1941 through March of 1944 it became the site for the German occupied Holocaust concentration camp imprisoning Romanian Jews.  As most concentration camps go, food, water, sanitation and disease outbreaks were common problems and concerns. Food was so limited that the prisoners were at one point fed barley bread that had 20% straw mix within and a species of pea known as Lathyrus sativus typically used to feed livestock.  It was only a short time later that strange symptoms began to break out amongst the prisoners. At first they became weak but it wasn’t long before they had difficulties ambulating and then became paralyzed with what was diagnosed as Neurolathyrism also known more simply as Lathyrism, a form of spastic paralysis. The culprit was oxalyldiaminopropionic acid (ODAP) from the peas. Some sources say that by 1943 hundreds of prisoners were struck down with Lathyrism and apparently 117 Jews were left permanently paralyzed.

We believe we first read about this in early 2013 in an online news article by Ronald Hamilton in a paper entitled “The Silent Fire: ODAP and the Death of Christopher McCandless”. McCandless has been made famous for his story and death deep in the Alaska wilderness in the Jon Krakauer book “Into the Wild”. We were excited to see this paper quoted in Jon Krakauer’s The New Yorker article this month. In his paper Hamilton wrote, 

 "… within months, hundreds of the young male inmates of the camp began limping, and had begun to use sticks as crutches to propel themselves about. In some cases inmates had been rapidly reduced to crawling on their backsides to make their ways through the compound …. Once the inmates had ingested enough of the culprit plant, it was as if a silent fire had been lit within their bodies. There was no turning back from this fire—once kindled, it would burn until the person who had eaten the grasspea would ultimately be crippled …. The more they’d eaten, the worse the consequences—but in any case, once the effects had begun, there was simply no way to reverse them …. “
In Krakauer’s recent The New Yorker article "How Chris McCandless Died”, he gives an explanatory full-circle synopsis regarding the gripping conclusion in his book “Into the Wild”.  In the article he speaks about Hamilton’s paper, McCandless’s fate and also mentions that in the 20th century more than a hundred thousand other people worldwide have been permanently paralyzed from eating grass pea containing the neurotoxin ODAP. According to Hamilton, the neurotoxin over-stimulates the nerve receptors causing them to burn out. As he explained in his “The Silent Fire” paper,
“It isn’t clear why, but the most vulnerable neurons to this catastrophic breakdown are the ones that regulate leg movement…. And when sufficient neurons die, paralysis sets in…. [The condition] never gets better; it always gets worse. The signals get weaker and weaker until they simply cease altogether. The victim experiences “much trouble just to stand up.” Many become rapidly too weak to walk. The only thing left for them to do at that point is to crawl….”
You can read the Krakauer / Hamilton account and recent story in the article link found below. In it they both tell the most recent events in the laboratory testing of the seeds that McCandless ate in bulk during his last months/weeks.  The tested seeds were found to be in high enough concentration to cause the symptoms (of Lathyrism) McCandless wrote about in his last weeks of survival when he was more susceptible to the neurotoxin having already been in severe malnutrition and hunger.

We dove a little deeper into this toxic myelopathy and discovered some helpful journal articles. According to Misra et al.  "patients complained of walking difficulty due to weakness and leg stiffness. The gait abnormalities ranged from spastic gait, toe walking and the need of assisted gait devises such as canes. They spoke of the weakness being mild to moderate and less prominent than was spasticity. In 8 of their subjects the physical signs were asymmetrical. Peripheral neuropathy was present in only one patient, but muscle atrophy and widespread fasciculation's were not found. A higher frequency of peripheral neuropathy and lower motor neuron involvement has been reported from Bangladesh and Israel. Severe spasticity in the absence of prominent weakness in lathyrism may be due to the involvement of certain specific groups of corticospinal fibres.“

We are big fans of Krakauer’s writing (and now Hamilton’s paper "The Silent Fire”) and this was a good story to close the Alexander Supertramp chapter hopefully once and for all. “Into the Wild” by Krakauer was a riveting book, one of our favorites.  Sean Penn’s cinematic interpretation of the story was good as well.  But for two gait geeks like us, to finally find out that poor Chris died of something so rare and complex, something that could be traced back as far as the holocaust concentration camps where people’s gait was first seen impaired was pretty fascinating. We are sure there billions of folks who have never looked at our blog and who will never likely read it.  Gait and gait related disorders and diseases do not get many people excited, but for us, this might as well be pornography. We are sure there are millions that are not fascinated by the fact that every organism on this planet locomotes in one manner or another, each with their own characteristic rules and biomechanics. It is sad to us that few really pay attention to how humans locomote and ambulate, at least not until they break a leg, sprain an ankle or come down with some complex motorneuron lesion. Gait is everywhere once you start looking for it. Whether you start to become aware of it at the local shopping mall, in TV commercials, during Sunday night football, during the Olympics, at your grandmama’s house, during your son or daughters walk to the dinner table , or even in the movies… . . gait is everywhere. Sean Penn’s interpretation of Krakauer’s “Into the Wild” is a touching sweeping movie and for us to now find out that the thing that ultimately led to McCandless’s demise was a gait related neurotoxic myelopathic disorder just goes to prove our point, gait is everywhere.  Why more people do not see this is beyond us, but hey, it would be a strange world if everyone was as nerdy as the two of us wouldn’t it !? 
Shawn and Ivo, The Gait Guys.  
 
References:
 
1. Paraplegia. 1993 Apr;31(4):249-54.

Clinical aspects of neurolathyrism in Unnao, India.

2. J Neurol. 2012 Jul;259(7):1263-8. doi: 10.1007/s00415-011-6306-4. Epub 2011 Nov 12.

Neurolathyrism: two Ethiopian case reports and review of the literature.

3. Neurobehav Toxicol Teratol. 1983 Nov-Dec;5(6):625-9.

Lathyrism: a neurotoxic disease.