George Huntington, On Chorea

Huntington

George Huntington (1850-1916) was a medical practitioner in Dutchess County New York.

His father and grandfather had both been doctors, and his family had lived on Long Island since 1797.

He gave his classic presentation, “On Chorea”, at the Meiga and Mason Academy of Medicine in Middleport Ohio in 1972, and this was subsequently published in the Medical and Surgical Reporter of Philadelphia on April 13, 1872.

This took place just one year after he graduated from his medical training at Columbia University.

He later explained his interest in the condition that now bears his name:

Over 50 years ago, in riding with my father on his rounds, I saw my first case of the “disorder”, which is the way the natives always referred to the dreaded disease.  I recall it as vividly as though it had just occurred but yesterday.  It made a most enduring impression on my boyish mind, an impression which was the very first impulse to my choosing chorea as my virgin contribution to the medical lore.  Driving with my father through a wooded road leading from East Hampton to Amagansett we suddenly came upon two women both bowing, twisting, grimacing.  I stared in wonderment, almost in fear.  What could it mean?  My father paused to speak with them and we passed on.  Then my Gamaliel-like instruction began; my medical instruction had its inception.  From this point on my interest in the disease has never wholly ceased.

Huntington’s disease (HD) is now know to be caused by a genetic mutation.  It is inherited in an autosomal dominant fashion, so that any child of an affected person typically has a 50% chance of inheriting the disease.  The mutation is a triplet repeat, so the genetics of HD lead to anticipation, a phenomenon whereby the disease may begin earlier and more severely in each successive generation.

HD can present at any age, but most often begins around 35-44 years with psychiatric disturbance such as depression or forgetfulness.  Neurological manifestations such as unsteady gait and the  jerky body movements (chorea) noted by George Huntington come later, followed by a progressive dementia.

Genetic testing is available, but pre-symptomatic testing for family members, who are at increased risk for the disease, is controversial since there is no treatment for HD.

Find out more about HD from the Huntington’s Disease Society of America.

Monmouth Neuroscience at the 2014 NJ Stroke Conference

We are happy to report that Monmouth’s stoke program was well represented at the 2014 New Jersey Stroke Conference earlier this month.

Two department of medicine residents, Drs Amor and Chan, presented our TIA center data in the poster session:

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Dr Holland gave a talk on the role of telemedicine in stroke rehabilitation.

Gamma knife tremor patient comes back to get the other side done!

Our first tremor patient treated with gamma knife radiosurgery recently came back, one year later, to have the other side treated.
He has essential tremor, which was affecting both arms.
He had undergone treatment to the left brain for right sided tremor last year.
He was so pleased with his results, he recently came to have the right brain treated to address the left sided tremor.
Here is his most recent video.
Note the action and postural tremor on the left (untreated) side, and the fact that he has almost not residual tremor on the right (treated) side.

Click here to find out more about Gamma knife radiosurgery for tremor at the Monmouth Neuroscience Institute.

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.

Nausea and vomitting, It’s not always what you think.

vomiting

Posted by Sara Ghotb, MD PGYIII Internal Medicine, Monmouth Medical Center

Case history:

This 74 y/o African American male presented to the emergency department with a 1-week history nausea ad intractable vomiting.

His past medical history included surgery for esophageal cancer in 2012 and hypertension.

He was admitted to the hospital for a gastrointestinal evaluation.  His symptoms did not improve with antiemetics and he also began to complain of headache and dizziness.  He was scheduled for an upper GI endoscopy.

What would you do next?

A.  Go ahead and do the upper GI endoscopy

B.  Order a brain imaging study

C.  Do a more detailed examination

D.  Prescribe more antiemetic medication