The brilliant and talented Robin Williams died in 2014. His death shocked the world, and it wasn’t until later that his family shared the results of his autopsy. Robin Williams’ brain was covered by the hallmark pathology of Lewy Body Dementia (LBD). This disease causes visual hallucinations, parkinsonian features (e.g. tremor and rigidity), and a fluctuating course (i.e. periodic exacerbations of symptoms followed by temporary improvements). Secondary symptoms include autonomous nervous system dysfunction (blood pressure fluctuations, urinary incontinence, constipation), REM sleep behavior disorders (e.g. acting out dreams), falls, and fainting spells.
LBD is caused by an abnormal build-up of Lewy bodies in the brain. Lewy bodies are made up of a protein—alpha synuclein. The distribution of Lewy Bodies in the brain results in changes in thinking, movements, and behaviors.
LBD is relatively less well understood by the general public compared to other forms of dementia. When most people hear dementia, they think Alzheimer’s disease. While Alzheimer’s disease is the most common type of dementia, there are unfortunately other types of progressive dementias that result in devastating changes in cognition and personality. LBD is differentiated from Alzheimer’s in several ways, mainly in symptom presentation. For example, short-term memory loss is more commonly an early symptom of Alzheimer’s, whereas problems with “executive functioning”, such as planning and problem solving, is more common in LBD. Sleep disturbances and well-formed visual hallucinations are also more common in LBD, whereas hallucination usually occur later in the course of Alzheimer’s disease. LBD also share features with Parkinson’s disease, particularly the motor symptoms, such as tremor and gait changes.
How is LBD diagnosed? Unfortunately, LBD is often misdiagnosed or not diagnosed until many years after symptoms have onset. In the case of Robin Williams, his diagnosis was not confirmed until after he died. There are no brain imaging studies or blood tests that can definitely diagnosis LBD. Neuropsychologists play a key role in the diagnosis, as they are uniquely equipped to perform the kind of cognitive testing that differentiates LBD from other dementias.
There is no cure for LBD, although research has led to a better understanding of which medications can help control the symptoms and which medications to avoid. Memory medications such as cholinesterase inhibitors, which were designed for use in Alzheimer’s patients, are also very effective in treating the cognitive changes in LBD. Other medications, such as traditional antipsychotics used to treat hallucinations in psychiatric patients, can have a detrimental effect on patients with LBD.
Proper diagnosis can help patients receive the best medications for symptom management and allow the patient and their family to cope with the disease.
Robin’s William’s widow recently published a letter about the difficult years leading up to her husband’s death. Read more here:
http://m.neurology.org/content/87/13/1308.full
Learn more about Lewy Body Dementia here:
