CJD Diagnostic Criteria

Diagnostic criteria have been created to confirm a diagnosis of definite, probable and possible CJD for each form of CJD. These criteria are constantly revised as research and experience provide more data. California physicians should note that Creutzfeldt-Jakob disease (CJD) and other transmissible spongiform encephalopathies (TSEs) are reportable diseases.

Criteria for Definite Sporadic Jakob-Creutzfeldt Disease
(WHO 1998)

  1. Diagnosed by standard neuropathological techniques
  2. AND/OR immunocytochemically
  3. AND/OR Western blot confirmed protease-resistant prion protein (PrP)
  4. AND/OR presence of scrapie-associated fibrils

Criteria for Probable Sporadic Jakob-Creutzfeldt Disease
(UCSF 2007)

  1. Rapid cognitive decline
  2. At least 2 of the following 6 symptoms:
    1. Myoclonus
    2. Pyramidal/extra pyramidal

CJD Differential Diagnosis

The disease course of CJD is highly variable and may mimic many other neurological disorders. The first step is to rule out alternative diagnoses.

A diagnosis of probable CJD requires an extensive exclusionary work up. The mnemonic device VITAMINS highlights other potential causes of rapidly progressive dementias:


It is important to rule out these conditions before confirming prion disease.


  • Brain angiogram, echocardiogram, carotid ultrasound

Rapidly Progressive Dementia Work Up

When you suspect a possible rapidly progressive dementia (RPD), there are certain tests you should order to help rule out or confirm a diagnosis. Many tests are needed for a complete work up, but a brain MRI including FLAIR (fluid attenuated inversion recovery) and DWI (diffusion weighted imaging) is the single most helpful tool in diagnosing CJD.

Physical exam and patient history

Share Your Story

Telling your story can help you feel better and connecting with others through similar experiences can provide comfort in knowing that you are not alone. Your story can also comfort those who knew the person before they became ill or others facing a similar experience.

Consider telling your story and connecting with others who are coping with Creutzfeldt-Jakob disease (CJD) or who have lost family members or friends to this terrible disease.

You can email us your story to include here on our site, or you can share your story on the Defeat Dementia Facebook Group.

Questions to Consider when Telling Your Story

  • How has caring for someone with prion disease affected your life? Your relationships? Your job or retirement plans?

Personal Stories

Read the stories of other CJD caregivers whose strength, spirit and courage helped them communicate with friends and family. Maybe someone has followed a similar path or found the humor or positive moment you need right now.


It all started in January of 1997.

My dad started feeling dizzy and was not sure what was going on. He was and had always been very healthy all his life. He worked at the Post Office and was still employed with them the day he passed. He had been there 35 years and never missed a day of work. NEVER.

Get Involved

Because Creutzfeldt-Jakob disease is a rare disease, many people have never heard of it. Educate others about what it is to be a caregiver and the need to find a cure for CJD.

Become a Participant

Treatments and cures for diseases are developed through research and clinical trials. If you want to help advance the work being done, participate in a quality treatment trial.

Become an Advocate

Find Support

A support group can provide a safe place to express your own needs and deal with painful emotions, including aggression, anger, mourning and guilt. It is a relief to know you’re not alone. One of the most valuable benefits of being in a support group is learning about coping skills and helpful resources from your peers. Every person's experience is so different, yet there are similarities that often only other caregivers can relate to. Many caregivers find new friends and social contacts in support groups.

Why a Support Group?

Preparing for the End

End-of-life plans can be scary to think about, but the speed of changes with rapidly progressive dementias make it essential to start planning as early as possible.

Legal, financial and health-related planning is important for all individuals, regardless of our health status. These documents define how you want to be medically cared for in case you are not able to speak for yourself. People often assume that their loved ones know their wishes about medical care but have never actually discussed it.

Participating in Research

Choosing to participate in research is an important personal decision, and we hope this section will help you better understand the research process. UCSF's Memory and Aging Center (MAC) is one of the few places in the world that conducts research and clinical trials of CJD. The CJD research at the MAC focuses on understanding prion disease, diagnosing it earlier and more accurately, and developing treatments for CJD.

What is clinical research?

Clinical research is generally considered to be a health-related investigation in human beings that follows a pre-defined plan. The UCSF Memory and Aging Center conducts both observational and interventional types of clinical research.

Hospice Care for CJD Patients

Hospice care provides palliative care to relieve suffering and improve the quality of life for people who are expected to live for about six months or less. People with CJD can often get into hospice earlier due to the rapid disease course. Like palliative care, hospice care can be provided whether you are at home or in an assisted living facility, nursing facility or hospital.

What is hospice care?

Hospice care provides palliative care to relieve suffering and improve the quality of life for people who are suspected to live for less than six months. People with CJD often qualify for hospice due to the rapid disease course. Like palliative care, hospice care can be provided at home, an assisted living facility, a nursing facility or a hospital.

Choosing a hospice care provider

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