Approach

There are no approved medicines for the treatment of Creutzfeldt-Jakob disease (CJD) or any prion diseases, so treatment is supportive. The information in this section is based on the experience of the CJD clinical team at the University of California, San Francisco (UCSF), Memory and Aging Center.

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Symptomatic management

There is no cure for prion disease due to its neurodegenerative nature. Drug treatment is targeted at symptom management and palliative care. Complications of immobility such as constipation, bowel obstruction, immobility, thromboses, pneumonia, decubitus ulcers, pulmonary emboli, and other conditions found in less ambulatory patients should be considered.

Mild to moderate anxiety and agitation

  • Clonazepam, lorazepam, or trazodone can be useful for treatment of anxiety and restlessness. Generally clonazepam is considered equipotent to lorazepam, but in clinical practice as little as 1.5 mg/day clonazepam has adequately replaced lorazepam 3 to 4 mg/day.

  • Selective serotonin reuptake inhibitors (SSRIs) such as citalopram can be tried, increasing dose quickly to effect.

Moderate to severe agitation or psychosis

  • Useful second-generation (atypical) antipsychotics include risperidone, olanzapine, quetiapine, and ziprasidone.

  • The practitioner may wish to avoid aripiprazole, which can be mildly stimulating.

  • If the patient is unable to take medications orally, haloperidol, olanzapine, or ziprasidone injections can be considered.

Myoclonus

  • Myoclonus causing distress to a patient or disturbing the ability to care for the patient is a sufficient indication for therapeutic intervention with benzodiazepines. Lorazepam and clonazepam are suitable options.

  • Valproic acid is often effective for the treatment of myoclonus; however, it is usually avoided when possible in early CJD or in patients undergoing experimental treatments, as one paper showed worse prion activity in vitro; whether valproic acid worsens prion disease in humans is not known.[140][141]

  • Other anticonvulsants such as levetiracetam and zonisamide have been used successfully to treat myoclonus in other conditions.

  • Myoclonus may also exacerbate existing pain conditions such as osteoarthritis.

Insomnia

  • Trazodone may be given at night as a sleep medication.

  • Clonazepam or lorazepam might also be helpful. If a benzodiazepine is currently being prescribed, the last dose is administered at or near bedtime.

  • Zolpidem and eszopiclone are alternatives. The US Food and Drug Administration (FDA) recommends that bedtime doses of zolpidem be lowered as data show that blood levels in some patients may be high enough the morning after use to impair activities that require alertness, including driving.[142]​ The data show the risk to be highest in patients taking the extended-release formulation, and that women appear to be more susceptible to this risk because they eliminate zolpidem more slowly from their bodies compared with men. The FDA also warns that eszopiclone can cause next-day impairment of driving and other activities that require alertness. As a result, the recommended starting dose has been lowered as higher doses are more likely to result in next-day impairment. The risk of next-morning drowsiness also applies to all drugs taken for insomnia, and the lowest dose that treats the patient's symptoms should be prescribed.

Depression

  • SSRIs can be helpful in treating depression in patients with prion disease. In addition to managing depression, they may be helpful in reducing anxiety, aggressive behaviour, obsessive compulsive behaviour, and poor impulse control.

  • Fluoxetine has a very long half-life and numerous drug interactions, so practitioners may want to avoid its use.

  • Paroxetine also has numerous drug interactions, but is an option.

Skeletal muscle pain

  • This is not a common symptom in sporadic CJD, and the aetiology should be evaluated.

  • Pain may be expressed as agitation in CJD patients who have impaired communication. Pain in CJD may be treated on a regular dosing schedule, and not as needed, due to the inability to communicate effectively.

  • In certain forms of CJD, such as variant CJD and rare sporadic CJD cases, pain may occur in regions of the body and may migrate; these syndromes are difficult to treat but would be treated in the same manner as neuropathic pain.[143]

  • Pain is treated with paracetamol, non-steroidal anti-inflammatory drugs (NSAIDs), or opioids, in that order of preference.

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