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MCID: SMN008
MIFTS: 47
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Semantic Dementia
Categories:
Mental diseases, Neuronal diseases, Rare diseases
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MalaCards integrated aliases for Semantic Dementia:
Characteristics:Orphanet epidemiological data:58
semantic dementia
Inheritance: Multigenic/multifactorial,Not applicable; Age of onset: Adult; Classifications:
ICD10:
33
Orphanet: 58
Rare neurological diseases |
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NINDS :
53
Frontotemporal dementia (FTD) describes a clinical syndrome associated with shrinking of the frontal and temporal anterior lobes of the brain. Originally known as Pick’s disease, the name and classification of FTD has been a topic of discussion for over a century. The current designation of the syndrome groups together Pick’s disease, primary progressive aphasia, and semantic dementia as FTD. Some doctors propose adding corticobasal degeneration and progressive supranuclear palsy to FTD and calling the group Pick Complex. These designations will continue to be debated. As it is defined today, the symptoms of FTD fall into two clinical patterns that involve either (1) changes in behavior, or (2) problems with language. The first type features behavior that can be either impulsive (disinhibited) or bored and listless (apathetic) and includes inappropriate social behavior; lack of social tact; lack of empathy; distractability; loss of insight into the behaviors of oneself and others; an increased interest in sex; changes in food preferences; agitation or, conversely, blunted emotions; neglect of personal hygiene; repetitive or compulsive behavior, and decreased energy and motivation. The second type primarily features symptoms of language disturbance, including difficulty making or understanding speech, often in conjunction with the behavioral type’s symptoms. Spatial skills and memory remain intact. There is a strong genetic component to the disease; FTD often runs in families.
MalaCards based summary : Semantic Dementia, also known as semantic primary progressive aphasia, is related to frontotemporal lobar degeneration with tdp43 inclusions, grn-related and prosopagnosia. An important gene associated with Semantic Dementia is PSEN1 (Presenilin 1), and among its related pathways/superpathways are Neuroscience and Alzheimers Disease. The drugs Memantine and Dopamine have been mentioned in the context of this disorder. Affiliated tissues include temporal lobe, brain and testes, and related phenotypes are aphasia and brain atrophy NIH Rare Diseases : 52 The following summary is from Orphanet , a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 100069 Definition Semantic dementia (SD) is a form of frontotemporal dementia (FTD; see this term), characterized by the progressive, amodal and profound loss of semantic knowledge (combination of visual associative agnosia, anomia, surface dyslexia or dysgraphia and disrupted comprehension of word meaning) and behavioral abnormalities, attributable to the degeneration of the anterior temporal lobes. Visit the Orphanet disease page for more resources. Wikipedia : 74 Semantic dementia (SD), also known as semantic variant primary progressive aphasia (svPPA), is a... more... |
Human phenotypes related to Semantic Dementia:58 31 (show all 11)
MGI Mouse Phenotypes related to Semantic Dementia:45
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Drugs for Semantic Dementia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):(show all 10)
Interventional clinical trials:(show all 16)
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MalaCards organs/tissues related to Semantic Dementia:40
Temporal Lobe,
Brain,
Testes,
Cortex,
Prefrontal Cortex,
Caudate Nucleus,
Amygdala
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Articles related to Semantic Dementia:(show top 50) (show all 1062)
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Genes related to Semantic Dementia (7 elite genes):(show all 11) - Elite gene
- Cancer Census gene in COSMIC
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Search
GEO
for disease gene expression data for Semantic Dementia.
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Cellular components related to Semantic Dementia according to GeneCards Suite gene sharing:
Biological processes related to Semantic Dementia according to GeneCards Suite gene sharing:(show all 24)
Molecular functions related to Semantic Dementia according to GeneCards Suite gene sharing:
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