PRION DISEASES (TSES)

Prion diseases (TSES) are a class of uncommon neurodegenerative disorders that may affect both animals and humans. Improperly folded proteins cause them in the brain, notably prion protein misfolding (PrP).

Creutzfeldt-Jakob disease (CJD) is the most frequent prion disease affecting humans. This causes a gradual loss in brain function, resulting in movement, behavior, and memory alterations. Prion diseases are ultimately lethal.

TYPES

Prion diseases (TSES) types include:

  • CJD
  • Variant CJD
  • Variably protease-sensitive prionopathy (VPSPr)
  • Gerstmann-Sträussler-Scheinker disease (GSS)
  • Fatal insomnia (FI)

SYMPTOMS

Prion diseases (TSES) have very prolonged incubation periods, frequently lasting several years. As symptoms appear, they intensify gradually, sometimes suddenly.

Common prion disease symptoms include:

  • Personality changes like depression, agitation, and apathy
  • Involuntary muscle spasms (myoclonus)
  • Trouble sleeping (insomnia)
  • Blindness or impaired vision
  • Difficulties with judgment, memory, and thinking
  • Disorientation or confusion
  • Loss of coordination (ataxia)
  • Slurred or difficult speech

DIAGNOSIS

Prion diseases (TSES) are diagnosed by sampling brain tissue during a biopsy or after death. Nevertheless, healthcare practitioners may do various tests before diagnosing prion disorders such as CJD or ruling out other diseases with similar symptoms. All persons with fast-advancing dementia should be evaluated for Prion diseases.

Among the tests are:

  • Fluid samples from the spinal cord (spinal tap, also called a lumbar puncture)
  • Blood tests
  • Electroencephalogram, a painless test that measures brain waves by placing electrodes on the scalp.
  • MRI (magnetic resonance imaging) scans of the brain
  • Neurologic and visual examinations to detect vision loss and nerve damage

TREATMENT

Currently, there is no cure for prion disease. Nevertheless, therapy focuses on giving comfort.

These are some examples of this kind of care:

  • Providing nutrients and hydration. A patient may need intravenous (IV) fluids or a feeding tube in more severe stages of the illness.
  • Assistance. As the condition progresses, many patients reach a point when they need assistance in taking care of themselves and completing activities of daily living.
  • Medications. Medications might be provided to relieve the symptoms. Here are several instances:

– Taking antidepressants or sedatives to alleviate psychological symptoms

– Taking opiate medicine to relieve discomfort

– Reducing muscular spasms with medications such as clonazepam and sodium valproate

The research for a cure that is also effective against prion diseases (TSES) is ongoing in the scientific community.

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