eMedicine Specialties > Neurology > Neurological Infections

Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy: Treatment & Medication

Author: Chitharanjan Rao, MD, DM, MRCP, DNB, Assistant Professor, Department of Neurology, University of Pittsburgh School of Medicine
Coauthor(s): Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Associate Program Director, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Contributor Information and Disclosures

Updated: Apr 27, 2007

Treatment

Medical Care

As is true for all prion diseases, no treatment is effective. However, some general principles apply, including the following:

  • Discontinue any medication that could impair cognition or cause confusion.
  • Many patients need psychiatric care, including antidepressants, which may provide a temporary relief.
  • Others need treatment to ameliorate sensory symptoms in the limbs.
  • All patients have increasing needs for supportive care, including palliative and terminal care.
  • The family members also need significant support in coping with the emotional and care needs. They also need reassurance and information regarding the nature of disease transmission.

Consultations

  • Neurologist
  • Infectious disease specialist
  • US Centers for Disease Control and Prevention
  • CJD surveillance unit, Edinburgh, United Kingdom

Diet

No dietary restrictions are necessary.

Activity

No activity restrictions are indicated.

Medication

A number of experimental interventions are currently being studied.86

They include some of the conventional medications such as the antimalarial quinacrine and the antipsychotic chlorpromazine, which prevent conversion of the normal prion protein (PrPc) to abnormal (PrPSc) prion protein according to in vitro studies.87 These drugs are currently being evaluated in treatment trials (PRION-1: Randomised trial of quinacrine in human prion disease).

Pentosan polysulphate (PPS) is another drug that has effects on prion protein production, replication, and associated cell toxicity. Some experimental results showed that if PPS is given to animals at a time relatively close to the point of experimental infection, then an increase in the incubation period of disease may occur; in some instances, animals appear to be completely protected from the development of disease.88 Based on these data, some individuals with prion diseases (the actual numbers and nature of prion disease are not known) have been treated with intraventricular PPS. Although the results are not known at this time, one patient with variant CJD reported has not shown any clear evidence of deterioration over a period of at least 23 months as of February 2005 (Potential Treatments for Creutzfeldt-Jakob Disease).

Another drug, flupirtine, has shown some beneficial effects on cognitive function in patients with CJD but without any evidence of increased survival with the treatment.89

One strategy involves designer compounds that interact with PrPSc structure, inhibiting the conformation change of PrPc associated with the disease.90

Another potential approach is immunologic, in which immunization with alpha s-helix peptides is used to reduce cerebral amyloid accumulation.91

More on Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy

Overview: Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy
Differential Diagnoses & Workup: Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy
Treatment & Medication: Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy
Follow-up: Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy
Multimedia: Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy
References
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Further Reading

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Keywords

transmissible spongiform encephalopathies, TSE, prion diseases, prionosis, prionoses, PrP diseases, Creutzfeldt-Jakob disease, CJD, sporadic CJD, sCJD, new variant CJD, nvCJD, variant CJD, vCJD, bovine spongiform encephalopathies, BSE, mad cow disease, mad cow, mad cows, scrapie, kuru, Gerstmann-Strãussler-Scheinker disease, GSS, familial fatal insomnia, FFI, sporadic fatal insomnia, SFI, chronic wasting disease, CWD

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Contributor Information and Disclosures

Author

Chitharanjan Rao, MD, DM, MRCP, DNB, Assistant Professor, Department of Neurology, University of Pittsburgh School of Medicine
Chitharanjan Rao, MD, DM, MRCP, DNB is a member of the following medical societies: American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, American Clinical Neurophysiology Society, American Medical Association, Medical Council of India, and Royal College of Physicians
Disclosure: Nothing to disclose.

Coauthor(s)

Florian P Thomas, MD, MA, PhD, Drmed, Director, Spinal Cord Injury Unit, St Louis Veterans Affairs Medical Center; Director, National MS Society Multiple Sclerosis Center; Associate Program Director, Professor, Department of Neurology and Psychiatry, Associate Professor, Institute for Molecular Virology, and Department of Molecular Microbiology and Immunology, St Louis University
Florian P Thomas, MD, MA, PhD, Drmed is a member of the following medical societies: American Academy of Neurology, American Paraplegia Society, and National Multiple Sclerosis Society
Disclosure: Nothing to disclose.

Medical Editor

Amy A Pruitt, MD, Program Director, Assistant Professor, Department of Neurology, University of Pennsylvania
Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Neil A Busis, MD, Chief, Division of Neurology, Department of Medicine, University of Pittsburgh Medical Center - Shadyside, Clinical Associate Professor, Department of Neurology, University of Pittsburgh School of Medicine
Neil A Busis, MD is a member of the following medical societies: American Academy of Neurology and American Association of Neuromuscular and Electrodiagnostic Medicine
Disclosure: Nothing to disclose.

CME Editor

Selim R Benbadis, MD, Professor, Director of Comprehensive Epilepsy Program, Departments of Neurology and Neurosurgery, University of South Florida School of Medicine, Tampa General Hospital
Selim R Benbadis, MD is a member of the following medical societies: American Academy of Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society, American Epilepsy Society, and American Medical Association
Disclosure: Nothing to disclose.

Chief Editor

Nicholas Y Lorenzo, MD, Chief Editor, eMedicine Neurology; Consulting Staff, Neurology Specialists and Consultants
Nicholas Y Lorenzo, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Neurology
Disclosure: Nothing to disclose.

 
 
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