Submitted Successfully!
Thank you for your contribution! You can also upload a video entry related to this topic through the link below: https://encyclopedia.pub/user/video_add?id=24574
Check Note
2000/2000
Ver. Summary Created by Modification Content Size Created at Operation
1 -- 1140 2022-06-28 13:08:45 |
2 format correct Meta information modification 1140 2022-06-29 03:29:56 | |
3 I have precised the type of SPECT and PET. I used diagnosis more than diagnostic + 10 word(s) 1150 2022-07-07 19:29:21 |
The Dementia with Lewy Bodies
Edit
Upload a video

Dementia with Lewy Bodies (DLB) is a common form of cognitive neurodegenerative disease. Only one third of patients are correctly diagnosed due to the clinical similarity mainly with Alzheimer’s disease (AD).

dementia with lewy bodies Alzheimer’s disease cerebro-spinal fluid MRI FP-CIT MIBG cognitive hallucinations RBD fluctuations
Information
Subjects: Neurosciences
View Times: 127
Entry Collection: Neurodegeneration
Revisions: 3 times (View History)
Update Date: 07 Jul 2022
Table of Contents

    1. Epidemiology of DLB

    Dementia with Lewy bodies (DLB) is the main neurodegenerative pathology affecting cognitive functions in the elderly after Alzheimer’s disease (AD) [1]. It begins after the age of 50 and represents 20% of demented patients [2]. First described in 1961 [3], it is only recently that it has been recognized as a common cause of dementia [4].

    2. Diagnosis Criteria

    DLB diagnosis criteria have evolved over time and were revised in 2005 and again in 2017 [5][6]. The McKeith criteria for the diagnosis of probable DLB are, in addition to cognitive decline (essential criterion), at least two core criteria and for the possible diagnosis either a core criterion with or without suggestive criteria, or at least one suggestive criterion (Table 1). McKeith and the international DLB consortium have also defined diagnosis criteria for prodromal forms [7]. The prodromal stage of DLB (pro-DLB), also called mild cognitive impairment due to Lewy bodies has been described in detail: the first criteria of this prodromal stage are similar to the stage of dementia with the difference that a decrease in functional capacity is either non-existent or minimal. So pro-DLB can be defined as the presence of the disease but cognitive impairment is not sufficient to lead to functional deficits in activities of daily living, and thus to dementia. Descriptions of pro-DLB criteria have been proposed [8]: pro-DLB can be defined as those patients who meet the revised diagnostic criteria for DLB but instead of dementia, fit the criteria for mild cognitive impairment (MCI) [7].
    Table 1. Diagnosis criteria of DLB.

    3. Differential Diagnosis with AD and PD

    Due to some clinical and neuropsychological similarities with AD, the diagnosis of DLB is not straightforward. The main confounding pathologies are AD, vascular dementia, Parkinson’s disease dementia (PDD), frontotemporal lobe degeneration (FTLD), progressive supranuclear palsy (PSP), multisystem atrophy (MSA), corticobasal degeneration (CBD) and prion diseases for brain diseases and recurrent depression, schizophrenia, bipolar disorders for psychiatric diseases. The most common differential diagnosis for the clinician remains with AD.
    DLB is close to Parkinson’s disease (PD) due to the presence of Parkinsonism (bradykinesia, rigidity, postural instability and resting tremor), which is often discrete, especially at the beginning of the disease [7][8]. DLB is also close to PD because of its pathophysiology, with the presence of positive α-synuclein (α-syn) aggregates in the brain, forming Lewy bodies [9]. Thus, PD and DLB, due to common aggregation processes, form a set of pathologies called synucleinopathies, including also PDD and multiple system atrophy (MSA). The pathophysiological difference that can be noted between DLB and PD is the aggregates localization. While α-syn aggregates are mostly localized in the brainstem and in the substantia nigra at the beginning of PD, they are rather diffuse throughout the brain in the early stages of DLB. Moreover, the three-dimensional structure of α-syn is different from the two others synucleinopathies: PD and MSA [10]. The differential diagnosis between PDD and DLB depends on the timing of events between the cognitive and motor impairment. In PDD, the cognitive symptoms appear after the parkinsonian syndrome, whereas in DLB the motor deficits appear after the cognitive deficits. Thus, according to an arbitrary rule, if the cognitive deficits appear at least one year after the motor deficits it will be PDD, otherwise it will be DLB [4][6].
    Many symptoms of DLB are close to those of AD, especially at the onset of the pathology: executive functions, visual memory, visuo-constructive and visuospatial abilities with weaknesses for episodic memory, short-term and working memory, verbal initiation, praxis, language, as well as social cognition [11]. DLB and AD can often be associated: 87% of DLB patients have moderate to abundant cortical amyloid plaques [12]. Thus, in these patients with AD/DLB diseases, DLB being masked, this disease is more difficult to diagnose.
    DLB presents certain clinical specificities such as visual illusions or hallucinations and fluctuations in attention, but also a particular sensitivity to neuroleptics [5][8]. Despite the very high diagnosis specificity of these criteria (the specificity of probable DLB is 95.1% in early stages and 88% in late stages) [4][13], their sensitivity remains low (32%) in pure DLB or even lower (12%) when associated with AD [14]. In other words, DLB is still a largely underdiagnosed disease in more than two-thirds of cases. It is therefore essential to discover new biomarkers that can distinguish DLB from AD to improve differential diagnosis.

    4. Therapeutic Management

    The treatment of DLB patients consists of a combination of pharmacological and non-pharmacological treatments. At the pharmacological level, each symptom has its own symptomatic treatment: donepezil or rivastigmine for cognitive disorders and cognitive fluctuations, levodopa in small doses for Parkinsonism, melatonin for behavioral disorders in REM sleep behavior disorders (RBD), clozapine, quetiapine and pimavanserine for invasive hallucinations or delusion [15]. Other antipsychotics are contraindicated because they are poorly tolerated: There is a risk of increased cognitive impairment, falls, increased parkinsonian syndrome, confusion, neuroleptic malignant syndrome and even death [16]. On a non-pharmacological level, cognitive remediation is used to combat cognitive disorders, and physical therapy to combat motor disorders and falls.

    References

    1. Zaccai, J.; McCracken, C.; Brayne, C. A systematic review of prevalence and incidence studies of dementia with Lewy bodies. Age Ageing 2005, 34, 561–566.
    2. McKeith, I.; Mintzer, J.; Aarsland, D.; Burn, D.; Chiu, H.; Cohen-Mansfield, J.; Dickson, D.; Dubois, B.; Duda, J.E.; Feldman, H.; et al. Dementia with Lewy bodies. Lancet Neurol. 2004, 3, 19–28.
    3. Okazaki, H.; Lipkin, L.E.; Aronson, S.M. Diffuse intracytoplasmic ganglionic inclusions (Lewy type) associated with progressive dementia and quadriparesis in flexion. J. Neuropathol. Exp. Neurol. 1961, 20, 237–244.
    4. McKeith, I.G.; Galasko, D.; Kosaka, K.; Perry, E.K.; Dickson, D.W.; Hansen, L.A.; Salmon, D.P.; Lowe, J.; Byrne, E.J.; Lennox, G.; et al. Consensus guidelines for the clinical and pathologic diagnosis of dementia with Lewy bodies (DLB): Report of the consortium on DLB international workshop. Neurology 1996, 47, 1113–1124.
    5. McKeith, I.G.; Dickson, D.W.; Lowe, J.; Emre, M.; O’Brien, J.T.; Feldman, H.; Cummings, J.; Duda, J.E.; Lippa, C.; Perry, E.K.; et al. Diagnosis and management of dementia with Lewy bodies: Third report of the DLB Consortium. Neurology 2005, 65, 1863–1872.
    6. McKeith, I.G.; Boeve, B.F.; Dickson, D.W.; Halliday, G.; Taylor, J.P.; Weintraub, D.; Aarsland, D.; Galvin, J.; Attems, J.; Ballard, C.G.; et al. Diagnosis and management of dementia with Lewy bodies: Fourth consensus report of the DLB Consortium. Neurology 2017, 89, 88–100.
    7. McKeith, I.G.; Ferman, T.J.; Thomas, A.J.; Blanc, F.; Boeve, B.F.; Fujishiro, H.; Kantarci, K.; Muscio, C.; O’Brien, J.T.; Postuma, R.B.; et al. Research criteria for the diagnosis of prodromal dementia with Lewy bodies. Neurology 2020, 94, 743–755.
    8. Donaghy, P.C.; McKeith, I.G. The clinical characteristics of dementia with Lewy bodies and a consideration of prodromal diagnosis. Alzheimer’s Res. Ther. 2014, 6, 46.
    9. Spillantini, M.G.; Schmidt, M.L.; Lee, V.M.; Trojanowski, J.Q.; Jakes, R.; Goedert, M. Alpha-synuclein in Lewy bodies. Nature 1997, 388, 839–840.
    10. Van der Perren, A.; Gelders, G.; Fenyi, A.; Bousset, L.; Brito, F.; Peelaerts, W.; Van den Haute, C.; Gentleman, S.; Melki, R.; Baekelandt, V. The structural differences between patient-derived alpha-synuclein strains dictate characteristics of Parkinson’s disease, multiple system atrophy and dementia with Lewy bodies. Acta Neuropathol. 2020, 139, 977–1000.
    11. Kemp, J.; Philippi, N.; Phillipps, C.; Demuynck, C.; Albasser, T.; Martin-Hunyadi, C.; Schmidt-Mutter, C.; Cretin, B.; Blanc, F. Cognitive profile in prodromal dementia with Lewy bodies. Alzheimer’s Res. Ther. 2017, 9, 19.
    12. Ballard, C.; Ziabreva, I.; Perry, R.; Larsen, J.P.; O’Brien, J.; McKeith, I.; Perry, E.; Aarsland, D. Differences in neuropathologic characteristics across the Lewy body dementia spectrum. Neurology 2006, 67, 1931–1934.
    13. Rizzo, G.; Arcuti, S.; Copetti, M.; Alessandria, M.; Savica, R.; Fontana, A.; Liguori, R.; Logroscino, G. Accuracy of clinical diagnosis of dementia with Lewy bodies: A systematic review and meta-analysis. J. Neurol. Neurosurg. Psychiatry 2018, 89, 358–366.
    14. Nelson, P.T.; Jicha, G.A.; Kryscio, R.J.; Abner, E.L.; Schmitt, F.A.; Cooper, G.; Xu, L.O.; Smith, C.D.; Markesbery, W.R. Low sensitivity in clinical diagnoses of dementia with Lewy bodies. J. Neurol. 2010, 257, 359–366.
    15. Verny, M.; Blanc, F. Lewy body dementia: Therapeutic propositions according to evidence based medicine and practice. Geriatr. Psychol. Neuropsychiatr. Vieil. 2019, 17, 189–197.
    16. McKeith, I.; Fairbairn, A.; Perry, R.; Thompson, P.; Perry, E. Neuroleptic sensitivity in patients with senile dementia of Lewy body type. BMJ 1992, 305, 673–678.
    More
    Information
    Subjects: Neurosciences
    Contributors MDPI registered users' name will be linked to their SciProfiles pages. To register with us, please refer to https://encyclopedia.pub/register : ,
    View Times: 127
    Entry Collection: Neurodegeneration
    Revisions: 3 times (View History)
    Update Date: 07 Jul 2022
    Table of Contents
      1000/1000

      Confirm

      Are you sure you want to delete?

      Video Upload Options

      Do you have a full video?
      Cite
      If you have any further questions, please contact Encyclopedia Editorial Office.
      Bousiges, O.; Blanc, F. The Dementia with Lewy Bodies. Encyclopedia. Available online: https://encyclopedia.pub/entry/24574 (accessed on 07 February 2023).
      Bousiges O, Blanc F. The Dementia with Lewy Bodies. Encyclopedia. Available at: https://encyclopedia.pub/entry/24574. Accessed February 07, 2023.
      Bousiges, Olivier, Frédéric Blanc. "The Dementia with Lewy Bodies," Encyclopedia, https://encyclopedia.pub/entry/24574 (accessed February 07, 2023).
      Bousiges, O., & Blanc, F. (2022, June 28). The Dementia with Lewy Bodies. In Encyclopedia. https://encyclopedia.pub/entry/24574
      Bousiges, Olivier and Frédéric Blanc. ''The Dementia with Lewy Bodies.'' Encyclopedia. Web. 28 June, 2022.
      Top
      Feedback