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

 

Cabin Fever

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Cabin fever  is a state of restlessness, depression and irritability brought on by an extended stay in a confined space or a remote and isolated area.

The term was first used to describe early U.S. settlers who experienced long winters snowed in alone in their log cabins – a well known example is the Montana winter of 1886-7 when snow fell every day from November through the end of February, cattle froze in place on the range, and ranchers ran short on coal, flour and wood.

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In 1915, polar explorer Sir Ernest Shackleton and his men made camp on a drifting ice floe in the so-called voyage of endurance for three months after their ship sank. The 28 men lived in crowded tents. Shackleton later wrote: “Day by day goes by, much the same as one another, We work; we talk; we eat.  …  The two subjects of most interest  …  our rate of drift and the weather.”

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The term has also applied to mental illness occurring on long oceanic voyages, after passengers and crew have endured long trips in small, cramped quarters below the deck of a ship

The mental anguish of cabin fever will often end in violence.  In 1959 a Russian at a Soviet Antarctic base murdered a colleague with an axe after losing a game of chess.  Following this, the Soviet authorities prohibited cosmonauts from playing chess!

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Probably the best known case of Cabin Fever affected author Jack Torrence in Stephen King’s novel and film, The Shining, while snow bound with his family in an old hotel.

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As the winter progresses, Jack becomes more and more unhinged, until he finally attempts to kill his wife and child.

What do you do if you think you are getting Cabin Fever (aside from trying to kill your wife an family)?  ……………  Get Out of the House if you can – for exposure to daylight exercise.  Maintain Normal Eating Patterns – try not to overindulge in junk food or skip meals altogether.  Set Goals – set daily and weekly goals, and track your progress toward completion.  Use Your Brain – although TV is a distraction, it is also relatively mindless, stimulating your mind can help keep you moving forward and reduce feelings of isolation and helplessness.

“Frozen Addicts” – An Unusal Twist on Drug-Induced Parkinsonism

The term “frozen addicts” was coined by Californian neurologist Dr William Langston after he had encountered an outbreak of akinetic rigid Parkinsonism in 6 drug users in Santa Clara County California:
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The story actually starts in 1947 with Hoffman-La-Roche chemist Dr Albert Ziering, who first synthesized synthetic opioid  Desmethylprodine 1,3-Dimethyl-4-phenyl-4-propionoxypiperidine (MPPP).  The drug was never developed or marketed.

The story continues with chemist Barry Kidston, who in the 1970s synthesized MPPP using Dr Ziering’s recipe and a home chemistry set.  However, a few days after injecting himself with a sample from a newly synthesized batch of drug, Kidston became frozen, unable to speak or walk.  He was taken to the hospital by his parents, misdiagnosed with catatonic schizophrenia, and treated with electroconvulsive therapy for months.  He was ultimately diagnosed him with Parkinson’s disease, and improved with L-dopa treatment.  Soon after, researchers analyzed the tainted drugs, and concluded that it was comprised of both MPPP and a similar compound, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).  Kidston died of cocaine overdose shortly afterwards.  His autopsy showed loss of dopaminergic cells in the substantia nigra, the hallmark of Parkinson’s disease.  The case was written up in the Journal Psychiatry Research in 1979.

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In July 1982, a 42 year old named George Carillo was hospitalized in San Jose frozen like a statue in a bent twisted position.

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Then a neurologist in Watsonville, only 30 miles away, reported 2 drug-addict brothers in their 20s  both with advanced symptoms of Parkinson’s.

Ultimately seven addicts came down with these same symptoms.

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A sample of tainted heroin was ultimately analyzed, and one of the toxicologists involved remembered reading about the Kidston case in Psychiatry Research.

William Langston, the neurologist who first treated Carillo, looked the case report up and found that Kidston had prepared drugs based on a 1947 paper by Albert Ziering.  But when Langston he went to the Stanford University library to read that original paper, he found that it had been cut out of the journal.  Some enterprising college chemist was cooking up MPPP and selling it as heroin, but like Kidston had made a mistake in his recipe and produced MPTP instead.

Once inside the brain, MPTP is metabolized into 1-methyl-4-phenylpyridinium which is toxic to dopamine producing neurons in the substantia nigra.

Since then MPTP has been used to develop an animal model of Parkinson’s disease, and this has allowed researchers to investigate surgeries to repair the injured region of the brain, new techniques using electrical stimulation, and more recently stem cell replacement of damaged cells.

These Frozen Addicts have also posed a question that we have yet to answer. If a street-drug impurity can trigger on form of Parkinson’s, could other “idiopathic” cases also have a chemical source? Recent studies have found that ingestion of the pesticide Rotenone can bring on Parkinson-like symptoms in mice. Loss of motor control, stiffening of muscles, and even loss of facial expression have been noted among the rare side effects of the high blood pressure medication, Reserpine, and the heartburn drug, Metoclopramide.

Neurology of Methamphetamine Abuse, “Feliz Breaking-Bad”!

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Methamphetamine was legally prescribed in injectable form in the US in the 1930-50s as a central nervous stimulant, antidepressant and appetite suppressant (for weight loss).

It became a widely abused prescription drug in the 1960’s, before it became more tightly regulated by the government in the 1970s.

That  is when illegal manufacturing and distribution really started to take off in the US.

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In the 1980s a new crystalline form of methamphetamine, which could be smoked, found it’s way into the US, and quickly started to replace cocaine as the illicit stimulant of choice among drug users.

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Whether snorted, smoked, or injected, methamphetamine rapidly crosses the blood brain barrier where they cause sustained increases in the extracellular concentrations of monoamine neurotransmitters such as  dopamine, norepinephrine, and serotonin.

With repeated use in both humans and experimental animal models, methamphetamine depletes the brain’s stores of monoamines,  contributing to methamphetamine’s high abuse potential – without the drug, users may have an impaired ability to experience pleasure (anhedonia), slipping into a deep depression

Dopamine and serotonin neurons project widely throughout the brain influence a variety of behaviors and functions. Up to 40% of chronic methamphetamine users have memory loss,  impulsive behavior and impaired decision-making.  Continued drug abuse can lead to depression and psychosis.


One interesting aspect of chronic methamphetamine psychosis is the delusion of parasitosis or formication – commonly known as “meth mites”, this is a frequent complaint in heavy daily users of methamphetamine, and can lead to self mutilation:

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AMC’s award winning TV drama Breaking Bad is a well-written and entertaining show that accurately depicts the consequences of moral turpitude and drug abuse:

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However, the show also had it’s lighter movements, here’s our Christmas tribute, Feliz Breaking-Bad or Christmas Meth!:

Malingering and Conversion Disorder, What’s the Difference?

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Both lead to mental or neurologic symptoms without any identifiable cause.

The difference is that while malingering is conscious and willful, conversion disorder is subconscious and involuntary.

What does this mean?

Well, malingering is deliberately feigning or exaggerating physical or mental symptoms motivated by a desire for financial compensation or avoiding work or military service:

One famous TV example of malingering was George Costanza, who faked a disability to get access to the executive bathroom:


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Another example, one of my personal favorites, is taken from Dirty Rotten Scoundrels.

Steve Martin, faking a disability to con money out of Glenne Headly, is tormented by competing con man Michael Cane who is pretending to be a doctor:

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In conversion disorder, or hysteria, the mental or physical symptom develops subconsciously in response to some stressful event or situation, and the affected patient truly believes they have a physical problem.

The Freudian theory suggests that a painful experiences is consciously repressed as a way of managing the pain, but this emotional charge is  somehow “converted” into the neurological symptoms.

In this scene from Talladego Nights, Ricky Bobby (Will Ferrell) emotionally traumatized by an accident, believes he is paralyzed.

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In this (long) clip taken from Speed Racer, Speed challenges a washed up racer to “wake up” his paralyzed R arm and race again:

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Finally, there many cases of both malingering and conversion disorder brought on by the stress of war in active duty military, and this recently declassified WW2 documentary from 1946 “Let there be light” shows examples of “Shell ShockedG.I.s undergoing some unconventional treatments.

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Mass hysteria is a particularly interesting social phenomenon where many people in one group together all share the same collective delusion of a disease, fear or exposure:


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Perhaps the most recent example of mass hysteria occurred in LeRoy New York in 2011-2012.   The 12 high school girls all developed Tourette-like symptoms , which led to extensive testing of their school for toxins.  The were all ultimately diagnosed with mass hysteria and conversion disorder:

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So, is there any concrete way to differentiate malingering from conversion disorder?

The answer is, maybe.

There are many clues gleaned from the physical exam that suggest a patient’s findings are non-organic, but this won’t tell you if the process is volitional or subconscious.

In a recent study, investigators compared PET scans from healthy individuals instructed to feign left arm weakness operating a joy stick, compared to controls who did the movement tasks normally.

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The feigners both had abnormal hypofunction of the right anterior prefrontal cortex not seen in the controls.

Perhaps a future objective test for malingering?

Rabies – The curse behind Vampires, Werewolves and Zombies?

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Rabies is a viral encephalitis transmitted to humans by the bite of an infected animal, usually a dog, fox or bat.

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The virus travels slowly along  peripheral nerves from the bite to the brain, and it can be several months between the animal bite and onset of the encephalitis.

Once encephalitis sets in, the disease is almost invariably fatal without immunization or post-exposure prophylaxis.

Symptoms include headache, fever, confusion and agitation, paranoia, terror, hallucinations, and delirium.  There is increased salivation, but attempts to drink or swallow lead to  excruciatingly painful spasms of the muscles in the throat and larynx leading to “hydrophobia” (fear of water).

The increased salivation, combined with unwillingness to swallow, leads to profuse drooling of saliva infected with virus.  The encephalitis leads to increased aggressiveness, unprovoked attack and biting, and thus facilitates the spread of the virus.

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Although the only confirmed cases human to human transmission of rabies have been recipients of infected donor organs, folklore has suggested transmission by sex, nursing and biting, inspiring stories about vampires, werewolves, vampires and zombies.

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The similarities between rabies and werewolves needs no further explanation.

In 1998, Juan Gomez-Alonso a Spanish neurologist wrote a paper in Neurology comparing vampirism with human rabies.

The most marked similarity are caused by rabid spasms of the head and throat.

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This leads to clenched teeth with retracted lips like and animal, and inability to swallow saliva with frothing at the mouth and vomiting of bloody fluid.

And the same goes for Zombies:

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These creatures first made fashionable in the 1954 book I am Legend, and then featured in the more recent movies 28 Days Later and World War Z, are even said to be caused by “infections” transmitted when a human is bitten by a demented zombie.

Click here to find out more similarities between Rabies, Vampires, Werewolves and Zombies.