CJD Creutzfeldt Jakob and Variant CJ Diseases

CJD Creutzfeldt Jakob and Variant CJ Diseases CJD and vCJD belong to a group of degenerative infectious neurologic disorders called transmissible spongiform encephalopathies. CJD- rare w no identifiable cause vCJD – is the human variation of bovine spongiform encephalopathy BSE (madcow disease) – results from eating meat that is infected from prions. Prions- pathogens smaller than a virus that are resistant to standard methods of disinfection and sterilization. CJD and vCJD both lack CNS inflammation. vCJD has shorter incubation than CJD . (<10 yrs) **Both diseases have progressive s/s , NO definitive treatments , and outcome is FATAL often within 1 year of symptom onset ** PATHOPHYSIOLOGY: Prion is unique bc it doesn’t have nucleic acid, which enables it to withstand conventional means of disinfection and sterilization. Prion crosses the BBB and is deposited into the brain tissue and causes degeneration of brain tissue. Cell death occurs and spongiform changes (spongy vacuoles) are produces in the brain. (look like little holes). The spongiform vacuoles are surrounded by amyloid plaque. There are 3 major forms of CJD 1. Sporadic CJD- 1 case per 1 million people 2. Familial or heridatary CJD 3. Acquired CJD – transmitted by contaminated brain, tissue, or neurosurgical instruments CLINICAL MANIFESTATIONS CJD and vCJD have different clinical features Psychiatric s/s occur early in vCJD , and late in CJD Age onset of vCJD 27 yrs, CJD 65yrs Presenting s/s of vCJD include affective s/s, sensory disturbances, and limb pain. Followed by muscle spasms, rigidity, dysarthria, incoordination, cognitive impairment, sleep disturbances CJD- present w mental deterioration, ataxia, and visual disturbances. Followed by memory loss, involuntary movement, paralysis, and mutism occur as the disease progresses. After s/s start vCJD survive an avg of 14 months, CJD survive <1 yr. ASSESMENT AND DX FINDINGS: Brain biopsy: ONLY way to confirm dx (but its not recommended) EEG, MRI , immunologic assessment – 3 dx tests currently used to dx CJD MEDICAL MANAGEMENT No effective tx for CJD or vCJD . Care is supportive and palliative . Goals include prevention of injury r/t immobility and dementia, promotion of pt comfort, and provision of support and education to family. NURSING MANAGEMENT Primarily supportive and palliative . Hospice services are appropriate at home or at an inpatient facility. PREVENTION is important- follow standard precautions. If sx is necessary , its recommended that disposable instruments be used and then incinerated, because conventional methods of sterilization wont work on the prions. If disposable instruments cannot be used, ,strict sterilization methods such as use of bleach for cleaning and extended time should be used.