Prion-Related Diseases Follow-up

Updated: Oct 27, 2014
  • Author: Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS; Chief Editor: Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM  more...
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Follow-up

Further Outpatient Care

The rate of progression of prion diseases is rapid. Frequent follow-up care is necessary to assess the need for symptomatic treatments. If the patient develops seizures, parkinsonian features, or behavioral problems, appropriate pharmacological treatments can be administered.

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Further Inpatient Care

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  • The initial steps of the dementia workup (eg, LP) can be performed most speedily if the patient is admitted to the hospital.
  • If the diagnosis is not clear, inpatient care speeds up referral for a brain biopsy.
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Deterrence/Prevention

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  • Prion diseases may spread by iatrogenic means. Hence, take care not to reuse EEG and/or electromyography (EMG) needles, surgical instruments, and other tools that have been exposed to a patient with prion disease. The prion agent is remarkably resistant to inactivation; hence, routine sterilization procedures, such as autoclaving, are ineffective.
  • As the incidence of BSE in Europe continues to decline, iatrogenic transmission from person to person is considered a serious threat to public health.
  • Preventive measures include donor deferral policies, technologies for prion removal from labile blood components and prion detection in plasma, and establishing a sensitive and rapid reference assay able to confirm the positive results from any putative blood screening assay. [135]
  • Interestingly, passive immunization with antibodies against prion protein (PrP), a major component of the prion infectious agents, was shown to protect mice from infection, indicating the possibility of prion vaccines. However, PrP is a host protein; therefore, immune tolerance to PrP has hampered development of them.
  • In the absence of a large-scale screening test, it is impossible to establish the prevalence of infection in the blood donor population and transfused patients. This lack of a test also prevents specific screening of blood donations. Since leukoreduction is probably insufficient to totally eliminate the transfusion risk, recently developed prion-specific filters could be a solution. [136]
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Complications

Any part of the CNS may be involved during the progression of prionoses; therefore, all types of CNS complications may be observed.

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Prognosis

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  • The prionoses are rapidly progressive. The median survival duration from the time of diagnosis to death varies from 8 months (as in sporadic CJD) to 60 months (as in GSS).
  • Patients with familial prion-related disease tend to have a longer course than those with sporadic disease.
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Patient Education

For excellent patient education resources, visit eMedicineHealth's Brain and Nervous System Center. Also, see eMedicineHealth's patient education article Mad Cow Disease and Variant Creutzfeldt-Jakob Disease.

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