You probably all have seen the latest on Robin Williams’s death. If not let me give you a synopsis, his wife, Susan Williams had an interview with ABC and confirmed that her husband’s death was not due to depression, is was due to Lewy Body Dementia. Lewy Body Dementia is the second most common dementia, with Alzheimer’s being the most common. Despite being the second most common dementia, it is the most misdiagnosed. There have been several cases along with Robin Williams’s that have shown that communities and doctors lack awareness and knowledge for this common misdiagnosed disease. Synder, wife of lawyer John Snyder who recently passed away due to Lewy Body Dementia, described the diagnosis of this disease like a game, “You’re playing wack a mole, getting symptoms you don’t understand and you’re going to doctors who don’t understand.” In this paper I will shed a bit of light to what Lewy Body Dementia is and why is it such a misdiagnosed disease.
Lewy Body Dementia was discovered in the 1900s when Friederich Lewy discovered abnormal protein deposits that caused abnormal function in the brain. These proteins are deposited in the brain stem. The brain stem contains 3 structures, the pons, midbrain and medulla oblongata. The midbrain contains something known as the substantia nigra (SN). The SN contains 2 structures, the pars reticula and pars compacta. Both of these are in charge of movement and the pars compacta itself also plays a role in temporal processing, that incudes sleep-wake cycle, and functions in the hippocampus, which is known to play a big role in our memory. The pars reticula contains GABAnergic neurons. In a normal brain, these neurons would release the neurotransmitter GABA to be able to increase the voltage of the dopaminergic neuron to then release dopamine. Dopamine is a very important neurotransmitter that plays a role in movement, our reward system and many other vital functions. In a brain with lewy bodies this is not possible because the protein deposits are “clogging up” this pathway, hence inhibiting dopamine. This is exactly what happens in Parkinson’s, Lewy Body Dementia though goes a step further. Instead of these Lewy Bodies staying in the brain stem only, they navigate to the cerebral cortex. This causes disruption in thinking, behaviors and perception. Alzheimer’s has a very similar physiology but with different types of proteins known as plaques, protein pieces called beta amyloid. On top of that, dead and dying nerve cells tangle together, both of these blocking cell to cell signal as well. As you can see these 3 diseases (Parkinson’s, Lewy Body Dementia, Alzheimer’s) are very similar in physiology but it is those small differences that help differentiate one disease from another, but because these differences are so small it can easy to not notice these small differences.
Lewy Body Dementia has a subset of symptoms. Impaired thinking, decrease in attention, visual hallucinations, movement problems, sleep disorders, behavioral symptoms, and changes in autonomic body function. Because there are so many symptoms and it has a very similar physiology to other similar disorders it can be present in various ways, and cause misdiagnoses. Lewy Body Dementia has 3 common appearances. It can start off with abnormal muscle movements, mirroring Parkinson’s. It can start off with dementia, leading to an incorrect diagnosis of Alzheimer’s and lastly it can start off with neuropsychological symptoms those being, hallucination and decrease in attention among the many, and lead to the correct diagnosis of Lewy Body Dementia. Unfortunately the last form of presentation of Lewy Body Dementia is the one that is least seen.
As we can see diagnosis is difficult in Lewy Body Dementia because it is misdiagnosed so much. So it is crucial to improve the diagnostic methods for this disease and diagnose as early as possible to be able to treat as early as possible. In order to diagnose Lewy Body Dementia a battery of test is performed. First a medical history including detailed lifestyle, medication list and prior surgeries and doctor appointments will be reviewed. Following that an MRI and CT scan, or at least one of those, will be performed. This will be done to be able to rule out any other disease that can account for the symptoms, like a brain tumor or cyst ect. Along with that blood test and other laboratory test will be performed. And lastly mental status and neuropsychological test will be performed to see the level of mental status of the patient. As you can see there is a lot of testing and imaging done, this is because there is not a conclusive way to diagnose Lewy Body Dementia, only till after death through an autopsy. That is why a group of doctors who specialize in different specialties is the best team to have for this disease.
Besides needing a comprehensive team of doctors for diagnosing it is also crucial to have a comprehensive team during the treatment phase. Current treatment for Lewy Body Dementia is medication. The reason for a comprehensive team of doctors is because not one medication works for majority of Lewy Body Dementia patients, not only that but many of the medications given to patients with Lewy Body Dementia can collide with others and cause a serious side effect. Because Lewy Body Dementia can present in 3 different types of ways, either with movement symptoms first, or dementia or neuropsychological symptoms, patients are given medication for what is presented first. Let say we have a 70 year old male patient, he comes presenting with dementia, he is given medication for dementia or Alzheimer’s. Because the patient in reality has Lewy Body Dementia but that isn’t known, because only the symptom of dementia is present, the patient may have a bad reaction to the medication because though he presents those symptoms of dementia that is not what he has. Same goes for when the doctors treat neuropsychological symptoms in this disease. Many anti psychotics do not work for patients with Lewy Body Dementia and if they do it can cancel out the patient’s medication for dementia and so forth.
This disease is definitely a puzzle and it is crucial to start researching methods for diagnosing this disorder as early as possible. Luckily recently, the University of Gothenburg has developed an international reference method for diagnosing Alzheimer’s that will be used as the standard worldwide. This will also benefit Lewy Body Dementia patients because the misdiagnosis between Lewy Body Dementia and Alzheimer’s will decrease because there is now a more concrete form of knowing if you have Alzheimer’s or not. This will also help in assisting scientist and doctors on how to create a better diagnostic method for Lewy Body Dementia because Alzheimer’s and Lewy Body Dementia have a similar physiology. Still, research in this disease is crucial to be able to give patients the best possible care.
University of Gothenburg. “Swedish diagnostic method for Alzheimer’s becomes international standard.” ScienceDaily. ScienceDaily, 2 November 2015. <www.sciencedaily.com/releases/2015/11/151102130138.htm>.
Diagnosis | Lewy Body Dementia Association.” Diagnosis | Lewy Body Dementia Association. N.p., n.d. Web. 15 Nov. 2015