Diagnosis and differential diagnosis of Parkinson’s disease
Jeremy Playfer, John Hindle, Andrew Lees in Parkinson's Disease in the Older Patient, 2018
Dementia with Lewy bodies (DLB) is characterised by a progressive dementia with a fluctuating course, extrapyramidal signs (EPS), visual hallucinations and increased sensitivity to neuroleptic drugs. Patients may present with cognitive impairment or with EPS. It is uncertain whether DLB is a distinct nosological entity or more likely representative of one part of the spectrum of Lewy body diseases that includes IPD. DLB should be diagnosed when dementia occurs before or concurrently with parkinsonism (if present). Dementia occurring one year after the onset of IPD has hitherto been classified as Parkinson’s disease with dementia (PDD) but the revised clinical criteria for DLB recognise difficulties in the strict application of this rule in clinical practice.130 There is, as yet, no clear agreement in the literature regarding a difference, if any, in the profile of the parkinsonian syndrome in DLB as compared with IPD; this may reflect bias in retrospective retrieval of data in autopsy studies as well as differing ‘sampling’ times during disease progression. The parkinsonian syndrome in DLB is more commonly a modest akinetic rigid syndrome without classical rest tremor. As well as PDD, the main differential is Alzheimer’s disease.
Introduction to dementia
Joanne Brooke in Dementia in Prison, 2020
Dementia with Lewy bodies (DLB) is sometimes known by other names, such as Lewy body dementia, Lewy body variant of Alzheimer’s disease, diffuse Lewy body disease and cortical Lewy body disease. Lewy bodies are named after Dr Friedrich Heinrich Lewy, a German-born neurologist. In 1912, two years out of medical school and in his first year as director of the Neuropsychiatric Laboratory at the University of Breslau (now Wroclaw, Poland) Medical School, he discovered ‘spherical neuronal inclusions’ in the brain of a deceased Parkinson’s patient (Sweeney et al., 1997). Lewy bodies are microscopic protein deposits in the brain associated with the death of brain cells. However, it is not known whether the Lewy bodies are the cause or effect of degeneration of brain cells, but they are thought to be the result of the misfolding of the protein alpha-synuclein (Gomperts et al., 2008).
Dementia and Lower Urinary Tract Dysfunction
Jacques Corcos, Gilles Karsenty, Thomas Kessler, David Ginsberg in Essentials of the Adult Neurogenic Bladder, 2020
Dementia with Lewy bodies (DLB) is the second most common degenerative cause of dementia.25 Lewy bodies are cytoplasmic inclusion bodies, and they appear to be widespread in the cerebral cortex and basal ganglia in patients with this disorder.26 Lewy bodies are α-synuclein-positive, presumably reflecting cytoskeletal alteration.27 In DLB, fluorodopa PET imaging reveals decreased dopaminergic neurons,28 while routine MRI scans are normal. In DLB, brain perfusion imaging by single-photon emission computed tomography (SPECT) shows diffuse decrease in perfusion including the occipital lobe (Figure 10.2), relevant to the visual hallucinations in this disorder. MIBG (metaiodo-benzylguanidine, a potent norepinephrine analog) myocardial scintigraphy shows decreased noradrenergic nerve terminals in the heart in patients with DLB. This is because DLB is a systemic disease affecting peripheral catecholaminergic autonomic fibers.
Clinical drug development for dementia with Lewy bodies: past and present
Published in Expert Opinion on Investigational Drugs, 2019
Garam Lee, Jeffrey Cummings, Boris Decourt, James B. Leverenz, Marwan N. Sabbagh
Dementia with Lewy bodies (DLB) is a devastating neurodegenerative dementia that remains an under-researched area. It is the second most common type of degenerative dementia after Alzheimer’s disease (AD), accounting for about 5% of all dementia cases [1,2], and the third most common of all the neurodegenerative diseases after AD and Parkinson’s disease (PD) [3]. DLB is associated with a poorer prognosis than AD with higher healthcare costs, greater caregiver burden, and a greater impact on quality of life [1]. Despite all this, DLB has received little attention and the majority of drug development for the dementias has been focused on AD in the past decades. There are currently no medications approved by the US Food and Drug Administration (FDA) for the treatment of DLB. Randomized controlled trials (RCTs) for DLB have only recently begun and the majority of studies in the past have been case studies or open-label observations with no controls [4,5]. Current pharmacological management strategies for DLB aim at providing symptomatic relief and involve medications approved for other indications. No disease-modifying therapies (DMTs) exist for DLB.
Systematic review of the pharmacoeconomics of Parkinson disease medications
Published in Expert Opinion on Pharmacotherapy, 2019
Alexander S. Wang, Steven A. Gunzler
There is a 24–31% point prevalence of PD dementia, which is associated with an increase in costs, rate of hospitalization, and nursing home admission [7,8]. With increasing age and duration of PD, a majority of people with PD will eventually develop dementia [8]. Dementia with Lewy bodies (DLB), sometimes considered a subtype of PD, causes diffuse cortical Lewy body pathology early in the disease, typically with cognitive impairment within one year of onset of parkinsonism, fluctuating cognition, and often visual hallucinations. Whereas rivastigmine has the most evidence for slowing progression of dementia in both PD and DLB, other acetylcholinesterase inhibitors and memantine are sometimes used in the treatment of PD dementia [8]. Hallucinations may occur, especially in PD dementia or DLB, and sometimes require antipsychotic medications or pimavanserin.
Neuro-ophthalmology of movement disorders
Published in Expert Review of Ophthalmology, 2018
Visual hallucinations, one of the most prominent features of dementia with Lewy bodies (DLB), are present early in the course of the disease, even before the onset of motor symptoms or before the introduction of dopaminergic medications. Abnormal saccades with impaired velocity, amplitude, and latency have been described in patients with DLB [52]. Progressive Balint syndrome that manifested by the triad of oculomotor apraxia, optic ataxia (the inability to accurately reach for something one is looking at), simultagnosia (inability to perceive more than one object at a time) was reported in a patient with DLB, few years prior to the onset of dementia, parkinsonism, and hallucinations [53]. Balint syndrome is usually associated with bilateral parieto-occipital structural lesions or dysfunction, and hypoperfusion in those areas was revealed on 99Tc-ECD-SPECT imaging in the patient with DLB. Retinopathy with degenerative changes in photoreceptors and some other retinal layers without Lewy body accumulation were reported in postmortem eye examinations of DLB subjects [32].
Related Knowledge Centers
- Dementia
- Behavior Change
- Cognition
- Dysautonomia
- Progressive Disease
- Activities of Daily Living
- Parkinson's Disease Dementia
- Lewy Body Dementias
- Prevalence
- Rapid Eye Movement Sleep Behavior Disorder