Prion-Related Diseases Follow-up

Updated: Dec 18, 2017
  • 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

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

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. [137]

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. [138]

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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

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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