A New Spin on The “Founder” of Neurology

Jean-Martin Charcot (1825-1893) is regarded by most scholars to be the founder of modern neurology.

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Known to be an excellent clinical teacjer, he was a professor at the University of Paris for 33 years and was  associated with Paris’s Salpêtrière Hospital that lasted throughout his life, ultimately becomiwas known as an excellent medical teacher, and he attracted students from all over Europe. His focus turned to neurology, and he is called by some the founder of modern neurology.

Charcot took an interest in hysteria, a mental disorder with physical manifestations, which he believed to be the result of an inherited weak neurological system, set off by a traumatic event like an accident

He learned the technique of hypnosis to evaluate these patients, and very quickly became a master of the relatively new “science.”

He believed that a hypnotized state was very similar to a bout of hysteria, and so he hypnotized his patients in order to induce and study their symptoms.

Charcot’s work also included other aspects of neurology – he was first to describe the degeneration of ligaments and joint surfaces due to lack of use or control, now called Charcot’s joint. He discovered the importance of small arteries in cerebral hemorrhage.  He described hereditary motor and sensory neuropathy.

He died in 1893 in Morvan, France.

The new movie focuses on his relationship with one hysterical patient named Agustine,

Click here to find out more about this.

2014, 100th Anniversary for Anosagnosia

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Anosagnosia

Here’s an illustrative example from a conversation with FD, an elderly woman who had had a right hemispheric stroke one week before, leaving her paralyzed on the left side and confined to a wheelchair:

How are you feeling today?

FD: I’ve got a headache.  You know, doctor, I’ve had a stroke so they brought me to the hospital.

Can you walk?

FD: Yes (FD had been in a wheelchair for the past week, and cannot walk)

Mrs D, hold out your hands.  Can you move your hands?

FD: Yes

Can you use your right hand?

FD: Yes

Can you use your left hand?

FD: Yes

Are both hands equally strong?

FD: Yes, of course they are equally strong.

Mrs D, point to me with your left hand.

FD: (Her hand lays paralyzed in front of her).

Mrs D, are you pointing to my nose?

FD: Yes

Can you see it pointing?

FD: Yes, it is about 2 inches from your nose.

Mrs D, can you clap?

FD: Yes, of course I can clap.

Mrs D, will you clap for me?

FD: (She proceeded to make clapping movements with her right hand, as if clapping with an imaginary hand near the midline)

Are you clapping?

FD: Yes, I am clapping.

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The term anosagnosia was first used by Joseph Babinksi on June 11, 1914 in a brief communication presented to the Neurological Society of Paris.

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He described two patients with left hemiplegia who didn’t know they were paralyzed.  The word comes from the Greek words nosos, “disease”, and gnosis, “knowledge”.

Affected patients deny their deficit, and overestimate their abilities, they state that they are capable of moving their paralyzed limb and that they are not different than normal people.

Their false belief of normality persists despite logical arguments and contradictory evidence – they may even produce bizarre explanations to defend their convictions.

If they admit any impairments, they will attribute them to other causes (i.e. arthritis, tiredness, etc.).

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But, this syndrome is not only seen with hemiplegia.

Visual anosognosia or Anton-Babinski syndrome is a rare neurological condition related to cortical blindness.  Affected patients deny their blindness and affirm adamantly that they are capable of seeing. The clinical presentation includes confabulations – instead of admitting blindness, they will make up answers when asked about what they see.

Mr Magoo is a great TV example of Anton’s syndrome – unaware of his loss of vision, he misinterprets and confabulates his way into trouble.

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Anosognosia may occur as part of receptive or Wernicke’s aphasia – affected patients cannot monitor and correct their own speech  errors and may appear angry and frustrated when the person they are speaking to fails to understand them.

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Watch this lecture, by Dr V. S. Ramachandran for more information on this fascinating syndrome:

Left world neglected

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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:

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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.

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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.

High altitude sickness and the size of your brain?

Posted by David Cuthbert, MSIV, Drexel University College of Medicine

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Every year thousands of people flock to several world locations with one common goal in mind – to push their bodies well beyond what nature has so far intended by climbing to dangerously high altitudes.

For whatever reason this appeals to some people.  Those people are eager to overcome the restrictions set by Mother Nature, despite the obvious dangers.

As a kid I loved the move Cliffhanger with Sylvester Stallone.  I remember watching that film and thinking that the only real dangers associated with extreme altitude were obvious – falling (please see video clip # 1), Slyvester Stalone wanting to use you as a human sled (watch video clip #1 again), the cold, or John Lithgow going crazy and wanting to use a helicopter to kill you for money (please see video clip #2).

But apparently those aren’t the only dangers, and medical school has taught me some pretty interesting stuff.

In fact there are a whole variety of medical syndromes that can occur at high altitude that don’t involve John Lithgow, and being that this is a neurology blog, this piece will focus on the neurological high altitude medical syndromes.  And in particular, will place emphasis on one terribly interesting study.

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Elevated intracranial pressure at high altitude may be a function of brain size?

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The “Tight Fit Hypothesis”, and the story of one extremely curious neurosurgeon.

The neurological syndromes of high altitude sickness are thought to be a spectrum of illness ranging from high altitude headache (HAH), to acute mountain sickness (AMS), to the most severe – high altitude cerebral edema (HACE).

The exact pathophysiologic mechanisms leading to these conditions are still somewhat unclear but several theories have been proposed and tested – all of which related to hypoxia and elevated intracranial pressure (ICP).

It is thought that in the milder end of this spectrum (HAH) the symptoms are solely contributed from hypoxia, and as disease severity progresses, and the patient comes closer to HACE – the pathogenesis is more attributable to raised ICP.

But what I find even more interesting than just the development of these syndromes – is the fact that there is great variability between who develops them.  For some people, no matter how acclimatized they are they simply cannot go to a certain altitude without great risk of HACE and subsequent death.  While others require little acclimatization, and are capable to trekking to the summit of Mt. Everest without necessitating the use of supplemental oxygen.  This leads one to ask, what are the factors present that allow someone this ability to tolerate high altitude?

One answer (with considerable evidence to support it) lies within their genetics.  The discovery of a transcription factor called “Hypoxia Inducible Factor”, or HIF, confirmed this.  HIF is a transcription factor that contributes to the regulation of several metabolic pathways, and allows both production of a higher concentration of hemoglobin, and greater sensitivity of the carotid body to hypoxia.  Another older, and forgotten theory looks to further explain this increased altitude tolerance through anatomic differences.

In 1985 Ross suggested that the “random nature of cerebral mountain sickness” can be explained by “more compliant systems”.  In other words, if a person has larger sized ventricles, and/or more atrophic brain, they will in turn be less susceptible to altitude sickness because the compliance will leave them better equipped to tolerate the raise intracranial pressure.

Interestingly enough, there existed someone crazy enough to test this hypothesis.  Someone not only willing to hike to these ungodly altitudes, but also willing to screw a bolt in his head to measure his own intracranial pressure.  This person was Brian Cummings, an avid outdoorsman, who also just so happened to be a neurosurgeon.

Cummings and a team of ten undertook an expedition to the Kishtwar region of northern India to try to put this hypothesis to the test.  However the data obtained from the experiment was destroyed in a fire.  Or so everyone thought!  Then recently Cummings wife (Cummings has since passed away) found the data from the experiment, allowing it to have since be published.

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Mountain rage within Kishtwar region of northern India.

In this experiment the “tight fit” hypothesis was tested by using 10 subjects.  These subjects had computed tomographic scans of their brains to measure their ventricular size.  After which a scoring system was used to measure symptomatology related to high altitude neurological syndromes while at high altitudes.  Also, three lucky volunteers had their intracranial pressures measured – allowed via screwing a pressure monitor through a burr hole in their heads .  This stayed in place while trekking through Northern India Himalaya’s.  Cummings himself participated in the study, and also had a pressure monitor placed.

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Dr. Cummings manipulating ICP monitor

The results of the study showed that the three subjects with the smallest ventricles suffered the most from Acute Mountain Sickness, and reported the worst headaches of the group.  Meanwhile patients with larger to normal sized ventricles reportedly had significantly less clinical findings related to AMS:

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Regarding the intracranial pressure monitoring, of the 3 subjects to be observed, one had large ventricles, one with normal size, and one with small ventricles.  The only one to experience headache, was the patient with the highest observed rise in ICP, and was also the subject with the smallest ventricles.

Therefore the results of this experiment support Ross’s “tight fit” hypothesis, and provide a potential anatomic explanation to compliment other genetic mechanisms to explain why some people are more prone to developing high altitude neurologic syndromes.

Obviously, the small study size cannot definitively explain this susceptibility, nor can it exclude other mechanisms as contributing as well. Nevertheless this experiment is considerably important to those who wish to conquer the hypoxic environment of Mother Nature’s higher altitudes.

It allows an explanation for those that are less able to adapt, and maybe even one day provide a means of testing their ability to acclimatize prior to their summit attempt.  And while that very well may never happen, this study at the very least is a great story about the incredible strength of the human spirit.

Dr. Cummings showed incredibly determination while searching for answers regarding the human ability to adapt to their environment, and fortunately now his work can live on.

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.

Memory misplaced

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Post written by Dr. Farida A. Malik , PGY3 Medical Resident, Monmouth Medical Center

Case Summary

This 69 year old lady had a remote history of breast cancer, hypertension and hypothyroidism.  She was brought to the Emergency Room by her husband because of abrupt onset confusion after waking up that morning. She was disoriented and was noted to ask the same questions over and over again. She had no difficulty walking, talking or dressing herself. She denied having headache or visual problems. There was no history of head trauma, seizures or any prior similar episodes.

When she was seen in the in the ER she knew her name and recognized her husband.  She was able to follow simple commands.  She had no recollection of events since morning or the day before. She repeatedly asked how she got to the hospital, despite being told several times that her husband brought her. Neurological examination otherwise was unremarkable.

CT scan of head, MRI of the brain and EEG were all normal.

She was diagnosed with TRANSIENT GLOBAL AMNESIA.

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The family was reassured about the benign nature of the condition and she was discharged home the next day still with memory lag.

Discussion

Transient global amnesia (TGA) is a clinical syndrome of reversible anterograde amnesia accompanied by repetitive questioning that occurs in middle-aged and elderly individuals.

The incidence of TGA is 5.2 to 10 per 100,000 per year overall, but 23.5 to 32 per 100,000 per year in adults aged 50 and over.

During a TGA episode recall of recent events simply vanishes. One may also draw a blank when asked to remember things that happened a day, a month or even a year ago.  Unlike “soap opera amnesia” (Jason Bourne) affected patients do remember who they are and recognize the people they know well.  But that doesn’t make their memory loss less any less disturbing.

Fortunately, episodes are usually short-lived, recover spontaneously, and are unlikely to recur.

The precise cause of TGA is unknown.  Atherosclerotic risk factors (eg. hypertension, diabetes, hypercholesterolemia) are not associated with TGA.
However there may be a link between TGA and history of migraines.

The primary site of neurologic functional disturbance is the medial temporal lobe and hippocampus.
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The pathogenesis of this transient disruption is unknown. Current theories include arterial ischemia, venous congestion, and migraine, but no theory explains all of the clinical features.

The diagnosis is made by the following signs and symptoms:

  • Sudden onset of memory loss, verified by a witness
  • Retention of personal identity despite memory loss
  • Normal cognition, such as the ability to recognize and name familiar objects and follow simple directions
  • Absence of signs indicating damage to a particular area of the brain, such as limb paralysis, involuntary movement or impaired word recognition
  • Duration of no more than 24 hours
  • Gradual return of memory
  • No evidence of seizures during the period of amnesia
  • No history of active epilepsy or recent head injury

Some common triggers identified are:

  • Sudden immersion in cold or hot water
  • Strenuous physical activity
  • Sexual intercourse
  • Medical procedures, such as angiography or endoscopy
  • Mild head trauma
  • Acute emotional distress, as might be provoked by bad news, conflict or overwork

There are no confirmatory diagnostic tests. The initial evaluation and management of patients with TGA focuses on excluding other diagnoses and should include the following:

  • If the patient is symptomatic on presentation, the patient should be observed in the hospital until the amnesia resolves.
  • Diagnostic testing includes oxygenation status, serum electrolytes, glucose, and a toxicology screen.

The need for further testing varies depending on the circumstances, such as how typical the event is for TGA, the presence of vascular risk factors, and whether the ictus was observed. Patients with recurrent or brief episodes, or activity suggesting motor automatism should be evaluated with EEG for possible epilepsy. A neuroimaging study may be performed in all patients, preferably a brain MRI with DWI, to exclude acute ischemia, head trauma, and other causes.

Treatment is not required for TGA. The condition usually does not recur, and the patient does not need to be restricted from driving unless events are recurrent.

There is no increased risk of mortality, epilepsy, or stroke following TGA as compared with age-matched controls.

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.