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Lewy body dementia(DLB)

MedGen UID:
199874
Concept ID:
C0752347
Disease or Syndrome
Synonyms: Autosomal dominant diffuse Lewy body disease; Diffuse Lewy body disease; DLB; Lewy Body Disease
SNOMED CT: LBD - Lewy body disease (80098002); Dementia of the Lewy body type (80098002); DLBD - Diffuse Lewy body disease (80098002); Cortical Lewy body disease (80098002); CLBD - Cortical Lewy body disease (80098002); Diffuse Lewy body disease (80098002); Lewy body variant of Alzheimer's disease (80098002); SDLT - senile dementia of Lewy body type (312991009); Senile Lewy body dementia (312991009); Senile dementia of the Lewy body type (312991009)
 
Genes (locations): GBA1 (1q22); SNCA (4q22.1); SNCB (5q35.2)
 
Monarch Initiative: MONDO:0007488
OMIM®: 127750
Orphanet: ORPHA1648

Definition

Dementia with Lewy bodies (DLB) is a neurodegenerative disorder clinically characterized by dementia and parkinsonism, often with fluctuating cognitive function, visual hallucinations, falls, syncopal episodes, and sensitivity to neuroleptic medication. Pathologically, Lewy bodies are present in a pattern more widespread than usually observed in Parkinson disease (see PD; 168600). Alzheimer disease (AD; 104300)-associated pathology and spongiform changes may also be seen (McKeith et al., 1996; Mizutani, 2000; McKeith et al., 2005). [from OMIM]

Additional description

From MedlinePlus Genetics
Dementia with Lewy bodies is a nervous system disorder characterized by a decline in intellectual function (dementia), a group of movement problems known as parkinsonism, visual hallucinations, sudden changes (fluctuations) in behavior and intellectual ability, and acting out dreams while asleep (REM sleep behavior disorder). This condition typically affects older adults, most often developing between ages 50 and 85. The life expectancy of individuals with dementia with Lewy bodies varies; people typically survive about 5 to 7 years after they are diagnosed.

REM sleep behavior disorder may be the first sign of dementia with Lewy bodies. It can occur years before other symptoms appear. Individuals with REM sleep behavior disorder act out their dreams, talking and moving in their sleep when they should be still. This behavior becomes less pronounced as dementia with Lewy bodies worsens and additional features develop.

Dementia is often the second major feature to develop in dementia with Lewy bodies. Initially, this intellectual decline may be mild or seem to come and go. In this condition, dementia often leads to impaired ability to perform visual-spatial tasks such as assembling puzzles. Affected individuals may also have poor problem-solving skills (executive functioning), speech difficulties, and reduced inhibitions. Problems with memory typically do not occur until later.

Most people with dementia with Lewy bodies experience visual hallucinations, which often involve people or animals. Fluctuations in behavior and thought processes (cognition) include sudden changes in attention, unintelligible speech, and brief episodes of altered consciousness that may appear as staring spells.

Parkinsonism is usually the last major feature to develop in people with dementia with Lewy bodies, although it can appear earlier in some individuals. The movement problems typically include tremors, rigidity, unusually slow movement (bradykinesia), and impaired balance and coordination (postural instability). Affected individuals may require walking aids or wheelchair assistance over time.

Individuals with dementia with Lewy bodies may also experience a sharp drop in blood pressure upon standing (orthostatic hypotension), fainting episodes (syncope), reduced sense of smell, increased saliva production and drooling, difficulty controlling the flow of urine (incontinence), or constipation.  https://medlineplus.gov/genetics/condition/dementia-with-lewy-bodies

Clinical features

From HPO
Delusion
MedGen UID:
3715
Concept ID:
C0011253
Mental or Behavioral Dysfunction
A delusion is a fixed false belief held despite evidence to the contrary. The term delusion broadly encompasses all false judgments that possess the following external characteristics to a significant, albeit unspecified, extent
Lewy bodies
MedGen UID:
43126
Concept ID:
C0085200
Cell Component
Intracytoplasmic, eosinophilic, round to elongated inclusions found in vacuoles of injured or fragmented neurons. The presence of Lewy bodies is the histological marker of the degenerative changes in LEWY BODY DISEASE and PARKINSON DISEASE but they may be seen in other neurological conditions. They are typically found in the substantia nigra and locus coeruleus but they are also seen in the basal forebrain, hypothalamic nuclei, and neocortex.
Visual hallucination
MedGen UID:
66688
Concept ID:
C0233763
Sign or Symptom
Visual perception in the absence of a visual stimulus.
Parkinsonian disorder
MedGen UID:
66079
Concept ID:
C0242422
Disease or Syndrome
Characteristic neurologic anomaly resulting from degeneration of dopamine-generating cells in the substantia nigra, a region of the midbrain, characterized clinically by shaking, rigidity, slowness of movement and difficulty with walking and gait.
Dementia
MedGen UID:
99229
Concept ID:
C0497327
Mental or Behavioral Dysfunction
A loss of global cognitive ability of sufficient amount to interfere with normal social or occupational function. Dementia represents a loss of previously present cognitive abilities, generally in adults, and can affect memory, thinking, language, judgment, and behavior.
Fluctuations in consciousness
MedGen UID:
377558
Concept ID:
C1851959
Finding
Changes in one's level of awareness and responsiveness to their environment.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLewy body dementia

Professional guidelines

PubMed

Taylor JP, McKeith IG, Burn DJ, Boeve BF, Weintraub D, Bamford C, Allan LM, Thomas AJ, O'Brien JT
Lancet Neurol 2020 Feb;19(2):157-169. Epub 2019 Sep 10 doi: 10.1016/S1474-4422(19)30153-X. PMID: 31519472Free PMC Article
Creavin ST, Wisniewski S, Noel-Storr AH, Trevelyan CM, Hampton T, Rayment D, Thom VM, Nash KJ, Elhamoui H, Milligan R, Patel AS, Tsivos DV, Wing T, Phillips E, Kellman SM, Shackleton HL, Singleton GF, Neale BE, Watton ME, Cullum S
Cochrane Database Syst Rev 2016 Jan 13;2016(1):CD011145. doi: 10.1002/14651858.CD011145.pub2. PMID: 26760674Free PMC Article
Stinton C, McKeith I, Taylor JP, Lafortune L, Mioshi E, Mak E, Cambridge V, Mason J, Thomas A, O'Brien JT
Am J Psychiatry 2015 Aug 1;172(8):731-42. Epub 2015 Jun 18 doi: 10.1176/appi.ajp.2015.14121582. PMID: 26085043

Curated

UK NICE Clinical guideline (CG148), Urinary incontinence in neurological disease: assessment and management, 2023

Recent clinical studies

Etiology

Petersen RC, Weintraub S, Sabbagh M, Karlawish J, Adler CH, Dilworth-Anderson P, Frank L, Huling Hummel C, Taylor A; Dementia Nomenclature Initiative
JAMA Neurol 2023 Dec 1;80(12):1364-1370. doi: 10.1001/jamaneurol.2023.3664. PMID: 37843871
Prasad S, Katta MR, Abhishek S, Sridhar R, Valisekka SS, Hameed M, Kaur J, Walia N
Dis Mon 2023 May;69(5):101441. Epub 2022 Jun 9 doi: 10.1016/j.disamonth.2022.101441. PMID: 35690493
Tolosa E, Garrido A, Scholz SW, Poewe W
Lancet Neurol 2021 May;20(5):385-397. doi: 10.1016/S1474-4422(21)00030-2. PMID: 33894193Free PMC Article
Cheng ST
Curr Psychiatry Rep 2017 Aug 10;19(9):64. doi: 10.1007/s11920-017-0818-2. PMID: 28795386Free PMC Article
Stinton C, McKeith I, Taylor JP, Lafortune L, Mioshi E, Mak E, Cambridge V, Mason J, Thomas A, O'Brien JT
Am J Psychiatry 2015 Aug 1;172(8):731-42. Epub 2015 Jun 18 doi: 10.1176/appi.ajp.2015.14121582. PMID: 26085043

Diagnosis

Devanand DP, Jeste DV, Stroup TS, Goldberg TE
Int Psychogeriatr 2024 Jan;36(1):28-42. Epub 2023 Mar 3 doi: 10.1017/S1041610223000157. PMID: 36866576
Prasad S, Katta MR, Abhishek S, Sridhar R, Valisekka SS, Hameed M, Kaur J, Walia N
Dis Mon 2023 May;69(5):101441. Epub 2022 Jun 9 doi: 10.1016/j.disamonth.2022.101441. PMID: 35690493
Taylor JP, McKeith IG, Burn DJ, Boeve BF, Weintraub D, Bamford C, Allan LM, Thomas AJ, O'Brien JT
Lancet Neurol 2020 Feb;19(2):157-169. Epub 2019 Sep 10 doi: 10.1016/S1474-4422(19)30153-X. PMID: 31519472Free PMC Article
Yousaf T, Dervenoulas G, Valkimadi PE, Politis M
J Neurol 2019 Jan;266(1):1-26. Epub 2018 May 14 doi: 10.1007/s00415-018-8892-x. PMID: 29761296Free PMC Article
Sanford AM
Clin Geriatr Med 2018 Nov;34(4):603-615. Epub 2018 Aug 21 doi: 10.1016/j.cger.2018.06.007. PMID: 30336990

Therapy

Devanand DP, Jeste DV, Stroup TS, Goldberg TE
Int Psychogeriatr 2024 Jan;36(1):28-42. Epub 2023 Mar 3 doi: 10.1017/S1041610223000157. PMID: 36866576
Rosoff DB, Bell AS, Jung J, Wagner J, Mavromatis LA, Lohoff FW
J Am Coll Cardiol 2022 Aug 16;80(7):653-662. doi: 10.1016/j.jacc.2022.05.041. PMID: 35953131
Li C, Liu J, Lin J, Shang H
Transl Psychiatry 2022 Jul 14;12(1):283. doi: 10.1038/s41398-022-02052-3. PMID: 35835752Free PMC Article
Connors MH, Quinto L, McKeith I, Brodaty H, Allan L, Bamford C, Thomas A, Taylor JP, O'Brien JT
Psychol Med 2018 Aug;48(11):1749-1758. Epub 2017 Nov 16 doi: 10.1017/S0033291717003257. PMID: 29143692Free PMC Article
Stinton C, McKeith I, Taylor JP, Lafortune L, Mioshi E, Mak E, Cambridge V, Mason J, Thomas A, O'Brien JT
Am J Psychiatry 2015 Aug 1;172(8):731-42. Epub 2015 Jun 18 doi: 10.1176/appi.ajp.2015.14121582. PMID: 26085043

Prognosis

Devanand DP, Jeste DV, Stroup TS, Goldberg TE
Int Psychogeriatr 2024 Jan;36(1):28-42. Epub 2023 Mar 3 doi: 10.1017/S1041610223000157. PMID: 36866576
Weintraub D, Aarsland D, Biundo R, Dobkin R, Goldman J, Lewis S
BMJ 2022 Oct 24;379:e068718. doi: 10.1136/bmj-2021-068718. PMID: 36280256
Martens YA, Zhao N, Liu CC, Kanekiyo T, Yang AJ, Goate AM, Holtzman DM, Bu G
Neuron 2022 Apr 20;110(8):1304-1317. Epub 2022 Mar 16 doi: 10.1016/j.neuron.2022.03.004. PMID: 35298921Free PMC Article
Tolosa E, Garrido A, Scholz SW, Poewe W
Lancet Neurol 2021 May;20(5):385-397. doi: 10.1016/S1474-4422(21)00030-2. PMID: 33894193Free PMC Article
Cheng ST
Curr Psychiatry Rep 2017 Aug 10;19(9):64. doi: 10.1007/s11920-017-0818-2. PMID: 28795386Free PMC Article

Clinical prediction guides

Forloni G
Int J Mol Sci 2023 Mar 21;24(6) doi: 10.3390/ijms24065914. PMID: 36982988Free PMC Article
Rosoff DB, Bell AS, Jung J, Wagner J, Mavromatis LA, Lohoff FW
J Am Coll Cardiol 2022 Aug 16;80(7):653-662. doi: 10.1016/j.jacc.2022.05.041. PMID: 35953131
Gate D, Tapp E, Leventhal O, Shahid M, Nonninger TJ, Yang AC, Strempfl K, Unger MS, Fehlmann T, Oh H, Channappa D, Henderson VW, Keller A, Aigner L, Galasko DR, Davis MM, Poston KL, Wyss-Coray T
Science 2021 Nov 12;374(6569):868-874. Epub 2021 Oct 14 doi: 10.1126/science.abf7266. PMID: 34648304Free PMC Article
Cheng ST
Curr Psychiatry Rep 2017 Aug 10;19(9):64. doi: 10.1007/s11920-017-0818-2. PMID: 28795386Free PMC Article
Stinton C, McKeith I, Taylor JP, Lafortune L, Mioshi E, Mak E, Cambridge V, Mason J, Thomas A, O'Brien JT
Am J Psychiatry 2015 Aug 1;172(8):731-42. Epub 2015 Jun 18 doi: 10.1176/appi.ajp.2015.14121582. PMID: 26085043

Recent systematic reviews

Elder GJ, Lazar AS, Alfonso-Miller P, Taylor JP
Int J Geriatr Psychiatry 2022 Oct;37(10) doi: 10.1002/gps.5814. PMID: 36168299Free PMC Article
Guay-Gagnon M, Vat S, Forget MF, Tremblay-Gravel M, Ducharme S, Nguyen QD, Desmarais P
J Sleep Res 2022 Oct;31(5):e13589. Epub 2022 Apr 2 doi: 10.1111/jsr.13589. PMID: 35366021
Arevalo-Rodriguez I, Smailagic N, Roqué-Figuls M, Ciapponi A, Sanchez-Perez E, Giannakou A, Pedraza OL, Bonfill Cosp X, Cullum S
Cochrane Database Syst Rev 2021 Jul 27;7(7):CD010783. doi: 10.1002/14651858.CD010783.pub3. PMID: 34313331Free PMC Article
Connors MH, Quinto L, McKeith I, Brodaty H, Allan L, Bamford C, Thomas A, Taylor JP, O'Brien JT
Psychol Med 2018 Aug;48(11):1749-1758. Epub 2017 Nov 16 doi: 10.1017/S0033291717003257. PMID: 29143692Free PMC Article
Stinton C, McKeith I, Taylor JP, Lafortune L, Mioshi E, Mak E, Cambridge V, Mason J, Thomas A, O'Brien JT
Am J Psychiatry 2015 Aug 1;172(8):731-42. Epub 2015 Jun 18 doi: 10.1176/appi.ajp.2015.14121582. PMID: 26085043

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2023
      UK NICE Clinical guideline (CG148), Urinary incontinence in neurological disease: assessment and management, 2023

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