VARIANT CREUTZFELDT-JAKOB DISEASE

Variant Creutzfeldt-Jakob disease (vCJD) is a prion disease or a form of transmissible spongiform encephalophaties, a family of few progressive neurodegenerative disorders. It affects both humans and animals. Patients with vCJD have distinguished psychiatric or frequent anxiety and depression or sensory symptoms. It delays the beginning of neurological abnormalities, such as ataxia, dementia, and myoclonus within weeks or months. This disease always leads to death. 

The duration of the disease is 14 months on average. It tends to affect younger individuals aged around 28 years old. The incubation period of vCJD after foodborne exposure is about ten years. The transmission of vCJD is through the consumption of cattle products that are contaminated with the agent of BSE. A cluster of misfolded prion proteins causes variant Creutzfeldt Jakob disease. It produces disease by disseminating the abnormal conformation to healthy cellular prion protein. The altering of normal prion to abnormal prion protein from a chain reaction leads to a higher content of particular forms of protein. 

TYPES

There are four types of Variant Creutzfeldt-Jakob disease:

  • Sporadic CJD – is the most common type but still very rare. The cause of sporadic CJD is unclear, but it’s claimed that a healthy brain protein changes abnormally or misfolds that turns in a prion. 
  • Variant CJD – is caused by consuming meat from a cow that is infected by a bovine spongiform encephalopathy, similar to prion disease in CJD. The incubation period is very long; it lasts for more than 10 years. It can also be transmitted by blood transfusion. 
  • Familial or inherited CJD – is a rare genetic condition where genes are inherited from their family ( parent) that carries a mutation, which causes prions to form in the brain during adulthood. 
  • Iatrogenic CJD – it is where the infection is fortuitously spread from someone with vCJD through a medical or surgical process. 

SYMPTOMS

The symptoms of Cruetzfeldt-Jakob disease depends on its type.  

Initial neurologic symptoms ( sporadic CJD) 

  • Dizziness
  • Vision problems
  • Difficulty in walking caused by coordination problems
  • Slurred speech

Initial psychological symptoms 

  • Severe depression 
  • Anxiety
  • Irritability 
  • Difficulty of sleeping 
  • An intense feeling of despair

Advanced neurological symptoms 

  • Loss of physical coordination
  • Muscle spasms
  • Loss of bladder control
  • Difficulty in swallowing 
  • Loss of speech

Advanced psychological symptoms 

  • Loss of memory 
  • Problems in concentrating 
  • Confusion
  • Loss of appetite 
  • Paranoia 
  • Aggressive behavior 
  • Inappropriate emotional responses

DIAGNOSIS

Diagnosing variant Creutzfeldt-Jakob disease is usually based on the medical history of a patient, symptoms, and some tests done. 

A doctor will rule out other conditions with similar symptoms like Parkinson’s disease, Alzheimer’s disease, or brain tumor by performing different tests. 

The diagnosis of vCJD is by examining the brain tissue by carrying out a brain biopsy. MRI, lumbar puncture, EEG, prototype blood test, tonsil biopsy, and genetic testing may also be done. 

TREATMENT

There’s no proven cure for Creutzfeldt-Jakob disease. But some treatments try to keep the patient comfortable by reducing symptoms with medicines. 

Psychological symptoms of CJD can be treated by antidepressants and sedatives. 

Muscle jerks or tremors an be treated by medicines like clonazepam and sodium valproate.

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