Lewy Body Dementia (LBD) is a debilitating neurodegenerative disease that is increasingly becoming a public health concern. "In Lewy body dementia, alpha-synuclein forms into clumps inside neurons, starting in areas of the brain that control aspects of memory and movement. This process causes neurons to work less effectively and, eventually, to die." Currently, 1 in 6 people are affected by a brain disease and totaling about 1.5 million people suffering nationwide by the disease, with an estimated 115 million cases by 2050. This public health concern is largely ignored, but it needs to be a priority. Accurate early-stage diagnostics will allow for more effective treatment, but this has been a difficult endeavor.
The Underlying Disease of a National Tragedy
"Diagnosing LBD can be challenging. Early Lewy body dementia symptoms are often confused with similar symptoms found in other brain diseases like Alzheimer's or in psychiatric disorders like schizophrenia." Affected people may suffer from symptoms that are unrelated, such as constipation, urinary difficulty, sleeplessness, and poor sense of smell. Some symptoms are more prevalent than others and worsen over time, and an observed prolonged sense of fear and anxiety can be an early indication of LBD. When an accurate and final diagnosis is made, it tends to occur at a relatively late stage of the disease.
Lewy Bodies not only affect the person who is suffering from the disease, but also have a tremendous emotional effect on loved ones. Susan Schneider Williams said of her late husband, Robin Williams, "I felt like he was drowning in his symptoms, and I was drowning along with him."
Touching Words from a Grieving Family
Dr. Doug Herthel was a renowned equine veterinarian who worked privately for President Ronald Reagan. He recently lost his battle with LBD. The progression of his disease was rapid and his family saw strange changes in his behavior. Doctors struggled to identify the issue and Dr. Herthel was misdiagnosed several times before LBD was confirmed. He became unable to drive or work and felt like a burden to his family. The stigma of a dementia diagnosis caused him to retreat from activities he loved. In addition to the physical symptoms, Dr. Herthel and his family suffered social isolation. His son, Mark, addressed the Alzheimer’s Disease-Related Dementias Summit to present "Insights from a Caregiver."
"His career was cut short by a diagnosis of Lewy Body Disease 17 months ago, a progressive and aggressive form of dementia. Doug was a brilliant problem solver and, in his honor, the Herthel family looks forward to supporting research focused on emerging treatment and, one day, a cure for Lewy Body Disease."
Ubiquitin: The Key to an Early and Accurate Diagnosis
Lewy Body Dementia is characterized by aggregations of alpha-synuclein. The cell recycles misfolded proteins by tagging the target with a chain of ubiquitin units that mark it for degradation by the proteasome. When this system is impaired, aggregations and plaques can form and kill neurons. The Ubiquitin Proteasome System (UPS) shows the earliest signs of dysfunction, long before symptoms manifest. The World Health Organization (WHO)recommends a focus on improving early diagnostics. LifeSensors is committed to finding early stage biomarkers for Parkinson’s Disease, Alzheimer’s Disease, ALS, Lewy Body Dementia, and Huntington’s Disease. Besides our internal R&D, LifeSensors provides innovative tools to researchers to study the UPS and its association with various pathologies. Our TUBE technology captures ubiquitylated proteins for the rapid identification of biomarkers.
The families of Robin Williams and Doug Herthel are pleading for accurate early stage diagnostics. LifeSensors is dedicated to making that happen by bettering the understanding of the UPS.