BRIDGEPORT, W.Va (WDTV) – Last week we learned about CJD, a prion disease. Tonight, on House Call, a medical professional from UHC explains how CJD is diagnosed and treated.
1). How is CJD diagnosed?
To diagnose CJD, a healthcare provider, typically a neurologist, will do a physical exam and ask about the person’s medical history. Depending on the findings, a doctor can use a few other tests to help diagnose CJD:
· A neurological exam can identify changes in reflexes, muscle twitching and spasms, poor coordination, and vision problems or blindness that may be signs of CJD.
· Electroencephalography (EEG) is a test that records the brain’s electrical patterns and can detect a specific type of abnormality in some types of CJD.
· MRI (magnetic resonance imaging) with diffusion weighted imaging creates detailed images of the brain using radio waves and a magnetic field and can detect changes in the brain that are common in about 90% of CJD cases.
· Cerebrospinal fluid (CSF) tests of the fluid that surrounds the brain and spinal cord include a newer test called Real-time quaking-induced conversion assay (RT-QulC). This is a much more specific test for prion disease and can detect the prions that cause CJD. CSF tests can also look for elevations in 14-3-3 and tau proteins. These proteins are non-specific markers of rapid brain cell death, which is seen in CJD as well as other neurodegenerative diseases. A lumbar puncture (also called a spinal tap) can collect CSF.
2). How is CJD treated?
Currently there’s no cure for CJD or any other prion disease, although researchers are testing some drugs to control CJD. Today’s treatments focus on making the person comfortable and easing symptoms. Medications may help relieve behavioral changes, seizures that may develop, and muscle jerks.
During later stages of the disease, people may need IV (intravenous) fluids and machine feeding. Because CJD and other prion diseases are incurable, these approaches can have limited use. Toward the end of life, people with CJD can benefit from hospice services.
3). What are the latest updates on CJD?
NINDS, part of the National Institutes of Health (NIH), is the nation’s leading federal funder of research on neurological disorders. NINDS supports new and innovative research to better understand, diagnose, and treat CJD. Other NIH institutes, including the National Institute of Allergy and Infectious Diseases, also conduct research on CJD.
Researchers are examining and characterizing the prions associated with CJD and other human and animal prion diseases. A better understanding of these diseases may help scientists discover factors that influence how prion changes infect nearby cells, how they are transmitted, and how the disorder damages the brain.
Researchers are also investigating how abnormal prion form and build up, as well as their reproduction in the brain.
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