TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHIES

Overview and Facts

Transmissible spongiform encephalopathies are an uncommon group of neurodegenerative disorders that can impact both animals and humans. They are induced by abnormally folded proteins in the brain, particularly prion protein misfolding. 

This causes a gradual decline in brain function, resulting in changes in memory, behavior, and movement. Transmissible spongiform encephalopathies are ultimately fatal. In the US, approximately 300 cases of TSEs are reported each year.

Misfolded PrP can connect to healthy PrP in people with transmissible spongiform encephalopathies, causing the healthy protein to fold abnormally as well. Misfolded PrP starts to build up and form clumps within the brain, causing nerve cells to be damaged and killed. Under a microscope, this damage induces tiny holes to form in brain tissue, giving it a sponge-like appearance.

Types and Symptoms

TYPES

  • Creutzfeldt-Jakob disease
  • Fatal Familial insomnia
  • Variant Creutzfeldt-Jakob disease
  • Gerstmann-Straussler-Scheinker syndrome
  • Kuru

SYMPTOMS

Transmissible spongiform encephalopathies have extremely long incubation periods, often lasting many years. When symptoms appear, they worsen gradually, sometimes rapidly.

Common prion disease signs include:

  • Problems with thinking, memory, and judgment
  • Disorientation or confusion
  • Depressions, agitation, or apathy
  • Lack of coordination
  • Uncontrollable muscle spasms
  • Insomnia
  • Blindness or impaired vision
  • Speech problems

Diagnosis & Medications

DIAGNOSIS

TSEs can be difficult to diagnose because their symptoms are similar to those of other neurodegenerative disorders.

A brain biopsy performed after death is the only way to confirm the diagnosis of transmissible spongiform encephalopathies. On the other hand, a healthcare professional can use your symptoms, health records, and a variety of tests to help diagnose the disease.

They may use the following tests:

  • Cerebrospinal fluid (CSF) testing
  • MRI
  • Electroencephalography

TREATMENT

There is no cure for transmissible spongiform encephalopathies at the moment. However, there are treatments that focus on providing comfort.

Here are some examples of this kind of care:

  • Medications

These can be prescribed to alleviate symptoms.

  • Using antidepressants or sedatives to alleviate psychological symptoms
  • Relieving pain with opiate medication
  • Relieving muscle spasms with medications such as sodium valproate and clonazepam

 

  • Support

As the disease progresses, many people require assistance in taking care of themselves and conducting daily activities.

  • Offering nutrition and hydration

This may be needed in the later stages of the condition.

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