I stride to honor the moemory of my father, Arturo de la Cerda.... Arturo was a well respected public servant (primarly having a career as of City Manager), throughout the great state of California. My dad was given a probable diagnosis of Sporadic Creutzfeldt-Jakob Disease (sCJD) in June of 2001, after all test for moreb treatable neurodgenrative diseases were ruled oot at the Greater Los Angles Regional VA Medical Ceter in colaberation with UCLA Neurology. Upon being told of the probable diagnosis, were told that the disease can hibernate for up to 6 years with subtle apperrences of symptoms. My dad's prodominate symptom was demntia. One notableinstance of the dementia was when he was nagociating a contract for the city of Corcoran, CA city council meeting and like the flick of a light switch he forgot where he was and what he was doing, luckly he was able to drive himself home that night. From that night, howevever more and isubtle symptom instancess of the dementia became more and more. apparent. Fortinatley during my dad's 4.5 mionth fight we were able to keep my dad home. It was not until the last month of his life was my mom no longer able to care for him and take him back to the regoional Los Angles VA hospital. After three weeks in the hospital my dad was than transfered to Golden State Convalecent Hospital only for a two night atay, taking his last breath on October 18, 2002...
What is Sporadic Creutzfeldt-Jakob Disease?
Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, rapidly progressive brain disorder caused by abnormal proteins called prions. It is the most common form of CJD and belongs to a group of diseases called prion diseases.
What does “sporadic” mean?
“Sporadic” means that the disease appears to arise spontaneously, rather than because of a known inherited mutation or a known exposure to contaminated tissue. In most cases, there is no identifiable cause or risk factor. Scientists think a normal prion protein can spontaneously change into an abnormal, misfolded form that then causes other proteins to misfold as well. Importantly, sporadic CJD is different from the “mad cow disease”–associated variant CJD. Eating beef is not considered the cause of typical sporadic CJD.
What happens to the brain?
The abnormal prion proteins accumulate and cause progressive damage to brain cells. Under a microscope, the brain develops characteristic changes sometimes described as “spongiform” because of the appearance of tiny vacuoles in brain tissue.
What are the symptoms?
The illness usually progresses very quickly, particularly compared with most forms of dementia. Symptoms can include:
Rapidly worsening memory and thinking problems
Personality or behavioral changes
Problems with balance and coordination (ataxia)
Involuntary muscle jerks (myoclonus)
Visual disturbances
Difficulty speaking or understanding language
Increasing confusion and loss of independence
The exact combination varies between people.
How common is it?
It is extremely rare—about 1–2 cases per million people per year worldwide. It most often occurs in later adulthood, with the peak incidence around the seventh decade of life.
Unfortunately, sCJD is almost always fatal, and the disease often progresses over months rather than years. A commonly reported median duration is around 4–6 months, although individual cases can vary considerably.
How is it diagnosed?
Doctors generally use a combination of:
Brain MRI, looking for characteristic patterns
Cerebrospinal-fluid (CSF) testing
EEG
A highly specific prion test called RT-QuIC (real-time quaking-induced conversion)
Neurological examination and the pattern/rate of symptom progression
RT-QuIC has substantially improved the ability to diagnose sCJD while a person is still alive.
There is currently no cure or treatment that stops the underlying disease, so treatment focuses primarily on comfort and managing symptoms.
