Locked in syndrome vs. coma

Coma can be caused by diffuse injury or dysfunction of the brain’s cerebral cortex or a by a lesion affecting the reticular activating system in the brain stem.  A comatose patient is unable to consciously feel, speak, hear, or move.

Brain death is a very severe form of coma with complete loss of brain function.  Once this has occurred, the affected patient is legally dead even though the heart, circulation and lungs may still be supported by artificial means. Patients classified as brain-dead can have their organs surgically removed for organ donation.

A patient with locked in syndrome can appear like they are comatose because they can’t move or speak, but they are aware and alert.   However, they can usually blink or move their eyes, and may be able to establish communication with others in this way.

There are numerous reported cases of patients with locked in syndrome after strokes or head trauma being misdiagnosed as comatose or even brain dead, some narrowly avoiding having their organs harvested.

Stroke Patient Hears Doctors Discuss Organ Donation

If you ever suspect a comatose patient may actually be locked in, you can try to establish communication with eye blinks, or get an EEG which (unlike coma) will be normal and reactive in locked in syndrome.

Patients with locked in syndrome can regain some quality of life:

This plight was made famous in the movie “The Diving Bell And The Butterfly” which was based on a memoir written by journalist Jean-Dominique Bauby.

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Jean-Dominique Bauby

 

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Cardiac and Concussion Screening at Monmouth March 15, 2014

MJM Flyer Spring Screening

Click here to find out more about concussion.

Click here to find out more about concussion screening.

Click here to find out more about the Matthew J Morahan Program at Barnabas Health.

Neurology and Skiing

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We’re mostly talking about orthopedic, spinal cord and head injuries.

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Spinal Cord Injuries:

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The incidence of spinal cord injury (SCI) is around 1 per 100,000 visits (mostly thoracolumbar spine), usually caused  caused by a simple fall, followed by hitting a tree.

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The incidence of SCI for snow boarders is higher, around 4 per 100,000 visits (mostly cervical), and usually caused by a backward fall during a jump (>75%) or other tick.

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Most SCIs in skiers and snow boarders occur in 15-25 years olds.

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Head trauma:

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The incidence of head trauma is 3.8 per 100,000 ski visits, usually from direct collision with trees.  The incidence of head trauma is higher for snow boarding, around 6.5 per 100,000 visits, more often caused by falling backwards.  Most head traumas result in concussions, but 14% are severe head trauma, and 4% are fatal.

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Others

I came across this case report of another unusual neurologic skiing complication: 
A 45-year old woman first developed distorted vision in the left eye, like looking through a kaleidoscope while skiing.  Soon afterwards, her right leg started jerking, causing her to fall. 
It turned out that she had sustained a left carotid artery dissection causing high grade stenosis during skiing.
There are a few other reports of cervical artery dissection from skiing.

Lawrence of Arabia and the Motorcycle Helmet

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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.

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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.

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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.

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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:

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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:

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

Concussion’s Axis of Evil

The term concussion is derived from the Latin word “concutere” which means “to shake violently”:

This term is used to describe a head injury associated with a temporary loss of brain function, including impaired consciousness, cognitive dysfunction and/or emotional problems.

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Concussion Center

To fully understand Concussion’s Axis of Evil, one need look no further than the brutal world of professional boxing and it’s neurological complications.

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One of the most savage beatings any fighter every received occurred on July 4, 1919 in Toledo, Ohio, when 24 year old Jack Dempsey destroyed 37 year old Jess Willard to become the Heavyweight Champion of the World.

One can easily spot the effects of concussion in Willard as he sustains blow after blow to the head, and he develops unsteady gait, erratic behavior (failing to avoid punches and protect himself) and ultimately unconsciousness.

New Jersey’s own Harrison S. Martland MD (1883-1954) was the first to report in 1928 that repeated beatings of this kind could lead to a delayed permanent neurologic syndrome referred to as punch drunk syndrome.

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His observations went largely unheeded.

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Muhammad Ali (born as Cassius Marcellus Clay in 1942) was only 22 when he became word heavyweight champion in 1964, almost 40 years after Martland’s paper was published.

Here is with Liberace in 1964:

Almost 10 years after that performance, Prof Corsellis reported further clinical and pathological features of punch drunk syndrome in his 1973 paper “The Aftermath of Boxing”.
Here’s data from one of his cases:

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By the 1980s, reports of abnormal brain CT scans in professional boxers had reached the popular media (Sports Illustrated, 1983):

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By 1983, Muhammad Ali was retired from professional boxing,

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and soon to be diagnosed with “Parkinson’s disease”.

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Here he is on the Today show with Bryant Gumbel in 1991:

Here he is in 2009:

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Obviously, repeated head trauma, and it’s consequences, is not unique to boxing:

concussion9John Grimsley (1962-2008) was a linebacker for the Houston Oilers.  He retired in 1993.  In 2008, aged 45, he was killed by an accidental gun shout wound to the chest.

His brain was examined as part of an ongoing study by Boston University’s Study of Traumatic Encephalopathy.

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concussion10His brain showed the same pathologic changes as the Punch Drunk boxers.

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This syndrome, more commonly referred to as Chronic Traumatic Encephalopathy, is now known to have occurred as a consequence of repeated head trauma in many other sports, including soccer, hockey, horse-racing and wrestling.

College football and amateur soccer players have been shown to have impaired performance on neuropsychologic testing, worse with increasing number of concussions.

Then, there’s the Second Impact Syndrome (SIS).

SIS is said to be a rare, often fatal, traumatic brain injury that occurs when a repeat injury is sustained before symptoms of a previous head injury have resolved.
Although limited to single case reports, and disputed as a discrete syndrome in the scientific literature, SIS cases are young athletes and have become high profile in the media:
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Click here to find out more about this case.

It has become clear that it takes athletes longer to recover from repeated that single concussions:
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This data, as well as SIS cases, has led to a concern that the presence of ongoing concussive symptoms are a significant risk factor for further injury to occur, and that any residual symptoms should mandate restriction for further contact sport in young athletes.

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Finally, it is know that concussions are under-reported by high school players.

A 2004 survey of 1500 varsity football payers in Milwaukee disclosed that although 15% had sustained a concussion during the season only 50% reported it to their coach or trainer.

So there we have it, Concussion’s Axis of Evil:

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And the solution?

The Allies Against Concussion:

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Click here to read more about how we have put these measures into effect at Monmouth Neuroscience Institute.

Click here to find out more about the Matthew J. Morahan III Health Assessment Center for athletes at Barnabas Heath.

Foreign Accent Syndrome – Their “Problem” or Yours?

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Foreign accent syndrome (FAS) is a rare condition which causes affected patients to suddenly speak their native language in a foreign accent.

Cases of FAS were reported as early as 1900.  However, one of the best known historical cases is “Astrid L”, a Norwegian woman who suffered a traumatic brain injury from shrapnel during a WW2 air raid in 1941.  She survived, but found herself mispronouncing vowels in such a way that she seemed to have a German accent, leading to social isolation and stigmatization for the remainder of the war.

Since then, there have been about another 60 FAS cases reported in the literature and media, mostly in patients who have suffered acute neurologic events such as strokes, multiple sclerosis and head injury.

Unlike most neurologic syndromes, FAS has not been localized to a lesion in a particular brain area.

The only thing that can be said is that most affected patients have lesions affecting the dominant hemisphere in or around known language areas.

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Brain imaging studies from a FAS patient: The MRI (left) shows enlargement of the Sylvian fissure from atrophy of the left temporal lobe. The PET scan (right) shows focal hypometabolism in the left temporal lobe.

Many affected patients were initially mute, then developed FAS as they recovered from a non-fluent aphasia:

There are also some cases of FAS that have developed after minor neurologic events, or even without any clearly identifiable neurological cause at all.  Some of these patients have had normal brain imaging, suggesting that the problem can be functional or psychogenic.

This is all further complicated by the fact that different listeners can perceive different accents in a single speaker.

The video clip is a patent with FAS syndrome after brain injury from hemiplegic migraine.  She is said to have a Chinese accent.  Does it sound Chinese or just slurred to you?

The table below is from a FAS case report, where the affected patient’s “foreign accent” was obviously described very differently by observers.

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This suggests that FAS may not be a true syndrome after all, but simply a listener-bound epiphenomenon.

What does this mean?

Well, we have already explained that most FAS patients have some kind of speech or language problem that changed how they speak.  That explains the association with lesions in the dominant hemisphere.   However the “foreign accent” may actually just something perceived by the listener – the variability of perceived accents is explained by the fact that listeners have different experiences with languages other than their own.

In other words FAS may not be a true syndrome, but simply an epiphenomenon that exists only in the ears of the beholder.

The advantages of a truly comprehensive concussion program

Newly published research questions the use of the computerized tool ImPACT in isolation for assessing concussion.

While ImPACT is useful for evaluating concussion overall, some parts of the rest can mistake normal controls as impaired almost half of the time.

This underscores the importance of using multiple assessments when evaluating concussion patients.

Another recent study shows that professional athletes are more likely to seek medical evaluation after sports concussion that university athletes.  Research published earlier this year already showed that high school athletes are least likely to be aware of the danger of concussions, and most likely to return to play too quickly.

All athletes referred to the Monmouth Neuroscience Concussion Center will get a truly comprehensive evaluation, including an evaluation by a neurologist, balance testing and the ImPACT test.