Tag Archives: Dr. Stanley Prusiner

What is CJD: Creutzfeldt-Jakob Disease?

1 Feb

Here is  a brief CJD FAQ, taken from UCSF’s Memory and Aging Center’s website. To learn more, visit the CJD website.

What is CJD?

Creutzfeldt-Jakob disease (CJD) is a human prion disease caused by a normal protein that becomes misshapen into a “prion”, builds up in the brain and disrupts normal brain function. It is a rare disorder that is fatal, usually within 6 months of diagnosis and a year of the first symptom. In the United States, there are about 300 new cases per year.

Creutzfeldt-Jakob disease (CJD) belongs to a family of diseases called “prion disease” [pree-ahn] caused by abnormal “prions”, microscopic infectious agents made of proteins. Prions cause a number of diseases in a variety of mammals, including bovine spongiform encephalopathy (BSE or “mad cow disease”) in cattle and scrapie in sheep.

What are the Signs and Symptoms?

Typical first symptoms include personality changes like anxiety and depression, memory loss, confusion and impaired thinking. Difficulties with balance and coordination as well as involuntary, jerking movements are also very common. People eventually lose the ability to move and speak. Pneumonia and other infections often occur in these patients and can lead to death.

What are the Tests?

When a diagnosis of Creutzfeldt-Jakob disease (CJD) is suspected, your doctor will likely order a number of tests. These can be helpful to either exclude other neurological diseases or support the CJD diagnosis.

Can I Participate in Research?

The CJD research at UCSF’s Memory and Aging Center focuses on understanding prion disease, diagnosing it earlier and more accurately, and developing treatments for CJD.

Other Resources

Please visit CJD Aware! a wonderful organization which provides information and support.

Get involved! Join the Defeat Dementia group on Facebook. Meet others in the fight against degenerative brain disorders such as Alzheimer’s disease (AD), non-AD dementias, including Frontotemporal Dementia and Creutzfeldt-Jakob disease (CJD) and others.

Here’s a link to the CJD video playlist from UCSF’s Memory and Aging Channel on YouTube.

Here’s a video featuring Dr. Michael Geschwind about the basics of Creutzfeld-Jakob Disease:

Mike Homer Has Died of CJD

1 Feb

I am sad to report that Internet industry legend Mike Homer has died today of CJD. This news was first reported this evening by Kara Swisher in her BoomTown blog. Kara writes:

We all liked Mike. In fact, we all loved the pugnacious, energetic and restlessly entrepreneurial Silicon Valley exec.

Sadly for those who knew him, Mike Homer died today at his home surrounded by family and friends, after a long battle with a severe illness. He was 50.

Homer is survived by his wife, Kristina, and three young children: James, Jack and Lucy.

His funeral is at Saint Raymond’s Catholic Church in Menlo Park on Thursday.

In 2007, Homer was diagnosed with Creutzfeldt-Jakob disease.

A rare, neurodegenerative “prion” disease, which in Homer’s case has occurred sporadically rather than via infection (the well-known variant that occurs in animals is called mad cow disease), CJD’s incidence is one case in a million annually, and few survive beyond a year after exhibiting symptoms.

His illness inspired his family and many friends to find treatments and a cure for the cruel disease and include the man–Dr. Stanley Prusiner–who won the Nobel Prize in 1997 for discovering prions, infectious agents that are at the heart of CJD.

Mike Homer was treated by specialists from UCSF. Homer’s fight made a tremendous impact on those who have been researching this devastating disease and raising public awareness about this and other forms of dementia.

Read about the Fight for Mike and Defeat Dementia campaigns here.

To learn more about current research and treatment options for CJD and other neurodegenerative diseases as well as how you can help, visit UCSF’s Memory and Aging Center’s CJD website or YouTube Channel.