Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy Treatment & Management

Updated: Feb 24, 2022
  • Author: Florian P Thomas, MD, PhD, MA, MS; Chief Editor: Niranjan N Singh, MBBS, MD, DM, FAHS, FAANEM  more...
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Approach Considerations

Although there is no cure for Creutzfeldt-Jakob disease (CJD), interleukins and other drugs may help slow the progression of disease. Medications to help treat the symptoms of CJD include antiepileptics to manage seizures (eg,diphenylhydantoin, carbamazepine, and gabapentin) and, occasionally, clonazepam to treat myoclonus. Antiepileptics are also used to treat violent outbursts. [131, 132, 133]

Dementia in patients with CJD can be treated with donepezil, galantamine, rivastigmine, and memantine. If a person develops disruptive behavior, antipsychotics can be used. Antidepressants may help patients come to terms with the prognosis.

Some general principles for the treatment of patients with variant CJD include the following:

  • Discontinue any medication that could impair cognition or cause confusion

  • Many patients need psychiatric care, including antidepressants, which may provide temporary relief

  • Some patients need treatment to ameliorate sensory symptoms in the limbs

  • All patients have increasing need for supportive care, including palliative and terminal care

  • Family members need significant support in coping with both emotional and care needs; they also need reassurance and information regarding the nature of disease transmission

Consultation with the following may be necessary:


Experimental Treatments

A number of experimental interventions are currently being studied. [134, 135] 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 prion protein (PrPSc), according to in vitro studies. [136] These drugs are currently being evaluated in treatment trials. [137, 138, 139] Clinical trials currently ongoing include PRION-1: Quinacrine for Human Prion Disease [138] and the CJD (Creutzfeldt-Jakob Disease) Quinacrine Study. [139] (Quinacrine is not commercially available in the United States.)

Pentosan polysulphate (PPS) also 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, an increase in the incubation period of disease may occur; in some instances, animals appear to be completely protected from the development of disease. On the basis of these data, some individuals with prion diseases have been treated with intraventricular PPS. One patient with variant CJD did not show any clear evidence of deterioration for a period of at least 23 months.

Flupirtine has shown some beneficial effects on cognitive function in patients with CJD but without any evidence of increased survival. [140] One strategy involves designer compounds that interact with PrPSc structure, inhibiting the conformation change of PrPc associated with the disease. [141]

Another potential approach is immunologic, in which immunization with alpha s-helix peptides is used to reduce cerebral amyloid accumulation. [142]