DEMENTIA, LEWY BODY

General Information (adopted from Orphanet):

Synonyms, Signs: DIFFUSE LEWY BODY DISEASE DIFFUSE LEWY BODY DISEASE WITH GAZE PALSY, INCLUDED
LEWY BODY DEMENTIA
LEWY BODY VARIANT OF ALZHEIMER DISEASE, INCLUDED
DLB
Number of Symptoms 8
OrphanetNr:
OMIM Id: 127750
ICD-10:
UMLs:
MeSH:
MedDRA:
Snomed:

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Autosomal dominant inheritance
[Omim]
Age of onset:

Disease classification (adopted from Orphanet):

Parent Diseases: No data available.

Symptom Information: Sort by abundance 

1
(HPO:0007159) Fluctuations in consciousness 1 / 7739
2
(HPO:0000726) Dementia 131 / 7739
3
(HPO:0000746) Delusions 21 / 7739
4
(HPO:0002367) Visual hallucinations 8 / 7739
5
(HPO:0001300) Parkinsonism 75 / 7739
6
(HPO:0000006) Autosomal dominant inheritance 2518 / 7739
7
(OMIM) Sensitivity to neuroleptic medication 1 / 7739
8
(OMIM) Diffuse Lewy bodies throughout the brain (cortical and subcortical regions) 1 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information:

Description: (OMIM) 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 ...
Clinical Description OMIM Ishikawa et al. (1997) reported 2 unrelated families with familial autosomal dominant diffuse Lewy body disease. In family A, 4 patients over 3 generations presented with parkinsonism, vertical ocular limitation, progressive dementia, and delusions or visual hallucinations. Two ...
Molecular genetics OMIM - SNCA Gene

Zarranz et al. (2004) reported a Spanish family with autosomal dominant parkinsonism and dementia with Lewy bodies, diagnosed using strict criteria. Neuropathologic examination showed diffuse distribution of Lewy bodies in cortical and subcortical ...