Prion Disease

Prion diseases or transmissible spongiform encephalopathies (TSEs) are a family of rare progressive neurodegenerative disorders that affect both humans and animals. They are distinguished by long incubation periods, characteristic spongiform changes associated with neuronal loss, and a failure to induce inflammatory response.
The causative agent of TSEs is believed to be a prion. A prion is an abnormal, transmissible agent that is able to induce abnormal folding of normal cellular prion proteins in the brain, leading to brain damage and the characteristics signs and symptoms of the disease. Prion diseases are usually rapidly progressive and always fatal.

In 1957 Kuru was the first human disease identified as a prion disease. Kuru was spread among the Fore tribe people in Papua New Guinea and caused by a ritual cannibalism. Besides the symptomatic coordinative malfunctions the cerebellar deficits were often associated by uncontrollable and inappropiate episodes of laughter, hence Kuru was named "laughing death" by the Fore people.

Creutzfeldt-Jakob Disease (CJD)

Diagnosed independently by Drs. Creutzfeldt and Jakob in the early 1920s, the disease that bears their names is a rare, degenerative brain disorder that affects approximately one person per million per year worldwide. This translates into 250-300 new U.S. cases annually. However, because Creutzfeldt-Jakob Disease (CJD) is very difficult to diagnose, this number may actually be higher. CJD affects men and women of all cultural backgrounds, with symptoms appearing most often in those aged 50 to 75.

Creutzfeldt-Jakob Disease is known as a prion disease, which means that healthy brain tissue deteriorates into an abnormal protein that the body cannot break down. CJD is a type of transmissible spongiform encephalopathy (TSE), a classification that includes both human and animal diseases. "Spongiform" refers to the characteristic appearance of an infected brain, which becomes filled with holes until it looks like a sponge under a microscope.
Prior to the "mad cow disease" epidemic of the 1990s, few people had heard of Creutzfeldt-Jakob Disease. However, once a growing number of young people in the United Kingdom started developing symptoms that appeared congruent with CJD, the world became alarmed. This new form of the illness, named variant Creutzfeldt-Jakob Disease or vCJD, results only from exposure to beef that is contaminated with bovine spongiform encephalopathy (BSE), the scientific term for mad cow disease.

There is not yet a cure for Creutzfeldt-Jakob Disease; only treatment for relieving symptoms. Current clinical trials are testing promising antiviral drugs such as amantadine, which has proved helpful in treating Parkinson's disease. Some patients taking these antivirals have shown brief periods of improvement, with no harmful side effects.

For more information on symptomes, etc:

http://www.helpguide.org/elder/creutzfeldt_jakob.htm

Plastic Surgery

Face Reconstruction

Although reconstructive plastic surgery is popularly believed to have been developed in the wars of the twentieth century, and particularly through the work of the East Grinstead Unit in the 1940s, reconstructive surgery has its origin in antiquity.

The particular area that received the most attention from early plastic surgeons was the nose. One of the first procedures for reconstructing the nose is attributed to Antonio Branca and is described by Heinrich von Pfolspeundt in his 1460 work 'Buch der Bündth-Ertznei'
Von Pfolspeundt illustrated a technique whereby a model of the nose to be reconstructed was made from parchment or leather. This model was then laid on the forearm and a line drawn around it. The marked area was then cut around and separated from the underlying layers in such a way that the bottom of the nose-flap remained attached to the arm. Subsequently the arm was raised to the head with the nose-flap positioned on the face and sutured to the defect.

The arm was then bound to the head to hold it in place. After 8-10 days when the skin had healed to the tissue of the defect, the lower part of the skin flap was cut, freeing the arm and allowing reconstruction of the nostrils (figure 1).
It was not until Gasparo Tagliacozzi (1546-99) wrote his seminal work 'De Curtorum Chirurgia per Insitionem Libri Duo' (On the surgery of defects by implantation) that the technique achieved widespread fame among European surgeons.

Surgeons had to wait until the late 18th century for the next significant advance to appear. Ironically the breakthrough came from rediscovering a procedure developed in ancient India. Its origins have been traced back to the 'Sushruta Samhita', ancient writings which date back to 8th century B.C. Among its 184 chapters was the technique using a leaf-shaped flap from the forehead to reconstruct the nose. This was published in the 'Gentleman's Magazine of Calcutta' in October 1794 and it soon became known as the 'Indian Method' (figure 2).

Figure 2 – illustration showing the skin flap used in the "Indian Method"

Following this, Felix Jean Casimir Guyon of Paris and Jacques Reverdin of Geneva reported the use of skin grafts in their practice in 1869. These grafts are still used widely today in reconstructive procedures.

Perhaps the most significant improvements in plastic surgical techniques however occurred in the last century. Innovative techniques such as the tubed pedicled graft developed during the Bolshevik revolution and World War I were refined by Archibald McIndoe and Harold Gilles who advanced the treatment of severe facial burns. These staged procedures rely on the growth and development of a blood supply from the recipient bed into the grafted tissue and may take many weeks or months (Figure 3–8).

Gillies wrote his 'Plastic Surgery of the Face' in 1920. In 1942, following the Battle of Britain, McIndoe performed his pioneering operations on burnt airmen at The Queen Victoria Hospital, pushing plastic surgery into the public eye. The "Guinea Pig" club was formed by those airmen who had been operated on by McIndoe and was the first patient support group for burns disfigurement. It is still going strong today.


Figure 3 & 4 – Patient of Harold Gillies with a facial defect











Figure 5 & 6 – Use of the tubed pedicled flap











Figure 7 & 8 – Post–operative result