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Prion-Related Diseases Follow-up

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

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

See the list below:

  • 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

See the list below:

  • 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

See the list below:

  • 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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Contributor Information and Disclosures
Author

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS Professor Emeritus of Neurology and Psychiatry, Clinical Professor of Medicine, Clinical Professor of Family Medicine, Clinical Professor of Neurosurgery, State University of New York Upstate Medical University; Neuroscience Director, Department of Neurology, Crouse Irving Memorial Hospital

Tarakad S Ramachandran, MBBS, MBA, MPH, FAAN, FACP, FAHA, FRCP, FRCPC, FRS, LRCP, MRCP, MRCS is a member of the following medical societies: American College of International Physicians, American Heart Association, American Stroke Association, American Academy of Neurology, American Academy of Pain Medicine, American College of Forensic Examiners Institute, National Association of Managed Care Physicians, American College of Physicians, Royal College of Physicians, Royal College of Physicians and Surgeons of Canada, Royal College of Surgeons of England, Royal Society of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Arun Ramachandran, MD State University of New York Upstate Medical University

Arun Ramachandran, MD is a member of the following medical societies: American Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Florian P Thomas, MD, PhD, Drmed, MA, MS Director, National MS Society Multiple Sclerosis Center; Professor and Director, Clinical Research Unit, Department of Neurology, Adjunct Professor of Physical Therapy, Associate Professor, Institute for Molecular Virology, St Louis University School of Medicine; Editor-in-Chief, Journal of Spinal Cord Medicine

Florian P Thomas, MD, PhD, Drmed, MA, MS is a member of the following medical societies: Academy of Spinal Cord Injury Professionals, American Academy of Neurology, American Neurological Association, Consortium of Multiple Sclerosis Centers, National Multiple Sclerosis Society, Sigma Xi

Disclosure: Nothing to disclose.

Chief Editor

Niranjan N Singh, MD, DM Associate Professor of Neurology, University of Missouri-Columbia School of Medicine

Niranjan N Singh, MD, DM is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Headache Society

Disclosure: Nothing to disclose.

Additional Contributors

Roberta J Seidman, MD Associate Professor of Clinical Pathology, Stony Brook University; Director of Neuropathology, Department of Pathology, Stony Brook University Medical Center

Roberta J Seidman, MD is a member of the following medical societies: American Academy of Neurology, Suffolk County Society of Pathologists, New York Association of Neuropathologists (The Neuroplex), American Association of Neuropathologists

Disclosure: Nothing to disclose.

Acknowledgements

The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors Thomas Wisniewski, MD and Einar M Sigurdsson, PhD to the development and writing of this article.

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Prion-related diseases. Spongiform change in prion disease. This section shows mild parenchymal vacuolation and prominent reactive astrocytosis.
Prion-related diseases. A representation of the human proteinaceous infectious particle, or PrP, gene. Mutations associated with inherited prionoses are shown above the gene, while polymorphisms are shown below the gene. A polymorphism at codon 129 (M versus V) is common in white populations, while a polymorphism at codon 219 (E versus K) is common in Japanese populations. The locations of the 4 putative helical regions, H1-H4, correspond to residues 109-122, 129-141, 178-191, and 202-218, respectively. This diagram does not illustrate all of the alpha-helical regions. A diagonal striped area represents the region of octarepeats, spanning residues 51-91. Octarepeats of 16, 32, 40, 48, 56, 64, or 72 amino acids at codons 67, 75, or 83 are indicated by the rectangle above the octarepeat region. These inserts are associated with familial Creutzfeldt-Jakob disease (CJD).
Shows characteristic signal changes of an MRI taken from a patient with sporadic CJD, using diffusion-weighted imaging (DWI). An abnormal signal is shown in both the basal ganglia (red arrows) and the cortical ribbon (yellow arrow).
Table 1. Prion-Related Diseases, Hosts, and Mechanism of Transmission
DiseaseHostMechanism
KuruHumanCannibalism
Sporadic CJDHumanSpontaneous PrPC to PrPSc conversion or somatic mutation
Iatrogenic CJDHumanInfection from prion-containing material, eg, dura mater, electrode
Familial CJDHumanMutations in the PrP gene
vCJDHumanInfection from BSE
GSSHumanMutations in the PrP gene
FFIHumanD178N mutation in the PrP gene, with M129 polymorphism
Sporadic fatal insomniaHumanSpontaneous PrPC to PrPSc conversion or somatic mutation
ScrapieSheepInfection in susceptible sheep
BSECattleInfection from contaminated food
TMEMinkInfection from sheep or cattle in food
CWDMule, deer, elkUnclear
Feline spongiform encephalopathyCatsInfection from contaminated food
Exotic ungulate encephalopathyNyala, oryx, kuduInfection from contaminated food
Table 2. Paraneoplastic Syndromes, Associated Tumors, and Autoantibodies
Clinical SyndromeNeoplasmAutoantibodies
Limbic encephalitisSmall cell lung carcinoma



Testicular/breast, thymoma



Anti-Hu, antiCV2,PCA-2, ANNA-3



Anti-Ma2 Anti-VGKC, anti-CV2



Cerebellar degenerationBreast, ovary, lung, othersAnti-Yo, anti-Ma, anti-Ri



Anti-Hu, anti-CV2



Opsoclonus myoclonusBreast, ovarian, small cell carcinoma of lung



Neuroblastoma



Anti-Ri, anti-Yo, Anti-Hu,



Anti-amphiphysin Anti-Hu



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