Lewy bodies dementia

Lewy body dementia (LBD, sometimes referred to as Lewy body disorder) is an umbrella term that includes Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB), two dementias characterized by abnormal deposits of the protein alpha-synuclein in the brain. Lewy body dementia (LBD, sometimes referred to as Lewy body disorder) is an umbrella term that includes Parkinson's disease dementia (PDD) and dementia with Lewy bodies (DLB), two dementias characterized by abnormal deposits of the protein alpha-synuclein in the brain. The synucleinopathies (DLB, PDD, and Parkinson's disease) have shared features of parkinsonism, impaired cognition, sleep disorders, and visual hallucinations. A core feature is REM sleep behaviour disorder (RBD), in which individuals lose normal muscle paralysis during REM sleep, and act out their dreams. Other frequent symptoms include visual hallucinations; marked fluctuations in attention or alertness; and slowness of movement, trouble walking, or rigidity. The autonomic nervous system is usually affected, and can result in symptoms such as changes in blood pressure, heart and gastrointestinal function, urinary incontinence, and constipation. Mood changes such as depression, apathy, and anxiety are common. RBD may appear decades before any other symptoms. On autopsy, 94 to 98% of individuals with polysomnography-confirmed RBD are found to have a synucleinopathy—most commonly DLB or Parkinson's disease, in about equal proportions. Other symptoms of the specific synucleinopathy usually manifest within 15 years of the diagnosis of RBD, but may emerge up to 50 years after RBD diagnosis. While both dementia with Lewy bodies and Parkinson's disease dementia have similar neuropathologic features, they are highly variable and should not be distinguished on pathologic features alone. Generally, DLB is distinguished from PDD by the time frame in which dementia symptoms appear relative to parkinsonian symptoms. DLB is diagnosed when cognitive symptoms begin before or at the same time as parkinsonism, while PDD is the diagnosis when Parkinson's disease is well established before the dementia occurs. Dementia with Lewy bodies and Parkinson's disease dementia are similar in many ways, suggesting there may be a common pathophysiological mechanism, with PDD and DLB at opposite ends of a LBD spectrum, and a shared component of protein deposits in Lewy bodies and Lewy neurites. Lewy bodies and Lewy neurites have been found to develop from aggregation of misfolded alpha-synuclein, a protein thought to assist in neurotransmitter release and vesicle turnover. However, no definitive link between Lewy bodies and neurodegenerative effects has been found. Despite differences in the timing of the appearance of symptoms, the two dementias 'show remarkably convergent neuropathological changes at autopsy'. Current disease management focuses on treating each symptom. For cognitive symptoms, the standard treatment is Cholinesterase Inhibitors (CHEIs). This drug class is effective in reducing apathy, visual hallucinations, and delusions symptoms. For movement symptoms, Levodopa is considered the standard treatment. Some research efforts in exploring alternative LBD therapeutics have been called questionable due to a variety of factors including choice of study design, exclusion criteria, limitation of outcomes, strength of evidence, and complications between PDD and DLB classifications. Beside pharmacological management, there are other available non-medical treatments like physical therapy, speech therapy, occupational therapy, individual and family psychotherapy, and support groups that might be recommended by physicians to improve and address the needs of each patient. As of 2013, Lewy body dementias affect about 1.3 million people in the United States, though this number is said to be an underestimate given the difficulty of diagnosing the disorder. LBD usually develops after the age of 50. While a study published on JAMA Neurology in 2013 pointed out a notable difference between DLB and PDD in age and sex, more research is needed to confirm any discrepancies. Lewy body dementias are more often misdiagnosed than any other common dementia. Most people with DLB had not heard of the condition prior to diagnosis; general awareness about LBD lags well behind that of Parkinson's and Alzheimer's diseases, even though LBD is the second most common dementia, after Alzheimer's. It is not only frustrating for families and caregivers to find that few people, including many healthcare professionals, are knowledgeable about LBD; lack of knowledge can have significant health consequences because people with LBD have severe sensitivity to antipsychotics often used to treat the symptoms. The Lewy Body Dementia Association (LBDA) and the Lewy Body Society promote awareness and provide support that helps society, by reducing costly use of healthcare, and families with LBD, by reducing stress. These organizations, and others in Argentina, Australia and Japan, help raise knowledge and help families with LBD become advocates to raise awareness about the disease. The British author and poet Mervyn Peake died in 1968 and was diagnosed posthumously as a probable case of DLB in a 2003 paper published in JAMA Neurology. Sahlas said his death was 'variously ascribed to Alzheimer disease, Parkinson disease, or postencephalitic parkinsonism'. Based on signs in his work and letters of progressive deterioration, fluctuating cognitive decline, deterioration in visuospatial function, declining attention span, and visual hallucinations and delusions, his may be the earliest known case where DLB was found to have been the likely cause of death.