George Huntington, On Chorea

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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.

Recent study links marijuana use to structural brain changes

 

 

Post  prepared by Amanda Baker, Drexel University College of Medicine Class of 2014

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A study recently published in the Journal of Neuroscience by Harvard researchers have linked casual marijuana use to structural changes in distinct areas of the brain.

These areas, the nucleus accumbens and amygdala, and are largely involved in recognition of reward, motivation, fear, and memory.  In this study, the brain scans of 20 young adult casual marijuana users were compared to those of 20 young adult non-users.

While the results clearly demonstrated significant structural differences between the two groups, the structural changes have not been correlated with consequences in mental or physical functioning.  In other words, researchers aren’t entirely sure of the impact of these brain changes.

 

Casual marijuana use may damage your brain

The debate regarding the use of marijuana medically and recreationally is ongoing in the United States.

Although the Drug Enforcement Administration(DEA) categorizes this drug as Schedule I, “with currently no accepted medical use and a high potential for abuse”, many argue that there is, in fact, much benefit to medical marijuana, especially in comparison to other sedating pain medications.

This is reflected in 21 state laws which have legalized medical marijuana to varying degrees.

Last summer, Dr. Sanjay Gupta completed a documentary “Weed” highlighting the benefit of medical marijuana:

However, some states such as Washington and Colorado, have gone one step further by legalizing marijuana for both recreational and medical use.

Given the ongoing research on the effects of marijuana, perhaps this new study will call into question continued legalization of the most widely used recreational drug in America.

 

Click here to find out more.

 

Limbic Encephalitis

Post prepared by Precious Ramirez-Arao, Monmouth Medical Center PGY3

A 60 year-old female was found lethargic lying in a pool of feces by roommate.

EMS was called and was immediately brought to the hospital.

In the emergency department she had a witnessed generalized tonic-clonic seizure.

Her roommate relates she had episodes of confusion and short-term memory loss over the past few weeks.

She remained lethargic over the next 72 hours in the hospital.

48-hour EEG monitoring showed diffuse 2 to 3 Hz delta slowing with periodic lateralized epileptiform discharges emanating from the left frontal temporal region.

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T2 weighted image of the brain showed signal abnormality of the left mesial temporal lobe and the pulvinar with diffusion restriction in the left hippocampus consistent with limbic encephalitis.

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Limbic encephalitis (LE) is a subacute syndrome of seizures, personality change and cognitive dysfunction, typically evolving over days to weeks.

Autoimmune and paraneoplastic forms have been described. The most common neoplasms associated with paraneoplastic LE are lung cancer (usually small cell), thymoma, ovarian or testicular teratoma, breast cancer and Hodgkin lymphoma. The associated autoantibody depends on the tumor type. Lung cancer and thymomas are associated with anti-VGKC while ovarian or testicular teratomas are associated with antiNMDA antibodies.

Neurologic symptoms can precede oncologic diagnosis for several months to years and initial CT scans are typically unrevealing.

Nevertheless, prompt and thorough evaluation for malignancy including PET and CT scan of the chest, abdomen and pelvis should be initiated. Symptomatic treatment includes corticosteroids, plasmapharesis and intravenous immune globulin.

 

Broca’s Aphasia vs. Aphemia

Aphasia is a disorder of speech an language caused by a strategic brain lesion.

Broca’s aphasia is a non-fluent type of aphasia with preserved comprehension caused by a lesion in the dominant (usually left) frontal lobe.

Broca’s affects both speech and writing. Because comprehension is spared, patients can monitor their own speech and become frustrated.

Affected patients will often find some alternate means of communication, other than speaking or writing, like Breaking Bad’s Hector Salamanca:

Aphemia is similar to Broca’s aphasia, but is caused by smaller lesions such that affected patients cannot speak but can still communicate with writing:

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

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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

Louis Pasteur’s New Jersey Connection

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We have already blogged about Rabies, and the paranoia invoked by this terrible almost invariably fatal illness.

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Part of the terror is the long incubation period between the bite of an infected animal and the onset of the disease, anywhere from 5d to 2 years later.

Before Louis Pasteur’s investigation and research in the late 1800’s, there was nothing a potentially infected victim could do except wait to develop this terrifying disease.

In the 1880s, Louis Pasteur and others proved that Rabies was an infectious disease that could be transmitted between species by infected saliva and blood.

They had also realized that Rabies was neurotrophic and targeted the spinal cord and brain. They could dissect an infected animal, remove the spinal cord, and infect other animals by the inoculation of this material.

Pasteur went on to find that strains of the virus become less lethal (“virulent”) when transmitted from dog to monkey or between other species, and that the virulence diminished with each transmission.

He also found that when sections of rabid spinal cord was suspended in dry air the virulence gradually diminished with time.

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This is how Pasteur produced the first attenuated vaccine, and successfully immunized 50 dogs against rabies.

Then on Monday 6 July 1885, Joseph Meister, aged nine, was brought to him from Alsace having been bitten by a rabid dog just 2 days before.

With some reluctance, Pasteur was persuaded by Drs Vulpian and Grancher of the Académie de Médecine to give Dr Grancher the emulsion from the cord of a rabbit that had died of rabies on 21 June and kept in dry air for 15 days, to innoculate the child.

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The boy was then given 13 further inoculations with samples form progressively fresher (more virulent) infected spinal cord over 10 days.  Pasteur waited every day for news of the boy from his doctos.  Finally when the boy had shown no signs of hydrophobis after three months, it seemed likely that the innoculations had averted the dreaded disease, and Pasteur announced that the vaccine had worked.

Not long after on the other side of the Atlantic in Newark (New Jersey) four boys had been bitten by a dog suspected to be rabid. A well-known physician, Dr. William O’Gorman had heard of Pasteur’s work with Meister, and recommended that the children be sent to him for treatment:

I have such confidence in the preventive forces of inoculation by mitigated virus that were it my misfortune to be bitten by a rabid dog, I would board the first Atlantic steamer, go straight to Paris and, full of hope, place myself immediately in the hands of Pasteur…. If the parents be poor, I appeal to the medical profession and to the humane of all classes to help send these poor children where there is almost a certainty of prevention and cure. Let us prove to the world that we are intelligent enough to appreciate the advance of science and liberal and humane enough to help those who cannot help themselves..

– New York Herald Tribune, December 4, 1885

This appeal to individuals in the United States, for those who desperately needed this medical treatment, as well as to philanthropists who recognized the global need to implement Pasteur’s new discoveries as standard medical procedure, created an uproar throughout the country.

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The four boys were sent to Paris accompanied by the one mother and a reporter.  Their story became a media sensation. It seemed that the entire nation was following the boys, who finally returned home in January 1886 cured.  The papers announced them as “heroes” are arranged for them to  tour and appear in major American cities.

The international publicity and donations resulting from this and other similar cases led to the foundation of the Pasteur Institute in 1888.  Patients came from all around the world for treatment.

By 1911,  the Philadelphia drug company H. K. Mulford was producing a rabies vaccine kit, using the Pasteur method, that could be shipped directly to doctors and was simple enough that “physicians who have had no previous experience may successfully apply it”.

three ampules of rabies vaccine, doses one, two, and three; 26 syringes with physiological salt solution; 26 needles for the syringes; two metal piston rods and two metal finger rests for the syringes; one two-dram vial of tincture of iodine; two charts for recording cases; one letter of general instructions; two stamped return envelopes; one record-of-treatment blank; and one vial of sterile wires. The treatment at this time had been reduced to only 21 doses to be administered one a day for 21 days

The kit contains three ampules of rabies vaccine, doses one, two, and three; 26 syringes with physiological salt solution; 26 needles for the syringes; two metal piston rods and two metal finger rests for the syringes; one two-dram vial of tincture of iodine; two charts for recording cases; and one letter of general instructions.

Watch the video below to find out more about rabies and post-exposure vaccination, which is 100% effective if administered right away:

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.