INFORMATION ON TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Transmissible spongiform encephalopathies also known as prion diseases are a group of rare degenerative brain disorders characterized by tiny holes that give the brain a "spongy" appearance. These holes can be seen when brain tissue is viewed under a microscope. It is a rare type of dementia that affects about one in every one million people each year.

Transmissible spongiform encephalopathies are a group of progressive conditions that affect the brain and nervous system of humans and animals and are transmitted by prions. Mental and physical abilities deteriorate and myriad tiny holes appear in the cortex causing it to appear like a sponge when brain tissue obtained at autopsy is examined under a microscope.

Transmissible spongiform encephalopathies cannot be transmitted through the air or through touching or most other forms of casual contact. However, they may be transmitted through contact with infected tissue, body fluids, or contaminated medical instruments. Normal sterilization procedures such as boiling or irradiating materials fail to render the pathogens non-infective.

SYNONYMS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Synonyms, Key Words, and Related Terms:

1 Prion diseases
2 Gerstmann-Sträussler-Scheinker diseases
3 Familial fatal insomnia
4 Sporadic fatal insomnia
5 Chronic wasting diseases

CAUSES OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

The cause of these diseases is unknown, but infected individuals and animals produce abnormal proteins which are laboratory markers used in the detection of infection.
The disease is thought to spread most commonly from ewe to offspring and to other lambs in the same group through contact with the placenta and placental fluids.
Human Transmissible Spongiform Encephalopathies can occur three ways: sporadically; as hereditary diseases; or through transmission from infected individuals
Sporadic TSEs may develop because some of a person's normal prions

SYMPTOMS OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Symptoms of Transmissible Spongiform Encephalopathies vary, but they commonly include personality changes, psychiatric problems such as depression, lack of coordination, and/or an unsteady gait. Patients also may experience involuntary jerking movements called myoclonus, unusual sensations, insomnia, confusion, or memory problems. In the later stages of the disease, patients have severe mental impairment and lose the ability to move or speak.
Progressive mental deterioration that soon leads to progressive unsteadiness and clumsiness, visual deterioration, and/or muscle twitching.

DIAGNOSIS TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

There are several tests that can be used to aid in the diagnosis of Transmissible spongiform encephalopathies. Specialized laboratories can analyze cerebrospinal fluid, blood and brain tissue obtained at biopsy or at autopsy

TREATMENT OF TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Transmissible spongiform encephalopathy tends to progress rapidly and usually culminate in death over the course of a few months to a few years.
Recent research from the University of Toronto and Caprion Pharmaceuticals have discovered one possible avenue which might lead to quicker diagnosis, a vaccine or possibly even treatment for prion diseases. The abnormally folded proteins which cause the disease have been found to expose a side chain of amino acids which the properly folded protein does not expose. Antibodies specifically coded to this side chain amino acid sequence have been found to stimulate an immune response to the abnormal prions and leave the normal proteins intact.