Rare diseases · Sign or symptom
Dementia
Dementia, progressive
HP:0000726
What it means
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.
In everyday care
Most people with this sign do not have a rare disease — it appears in common conditions far more often. These general reference pages cover the same topic:
Rare diseases that can present with this104
Very common80–99%
16- ABeta amyloidosis, Dutch type
- ABeta amyloidosis, Iowa type
- ABetaL34V amyloidosis
- Amelocerebrohypohidrotic syndrome
- Autosomal recessive cutis laxa type 2, classic type
- Autosomal recessive spastic paraplegia type 21
- Early-onset autosomal dominant Alzheimer disease
- Fatal familial insomnia
- Fragile X-associated tremor/ataxia syndrome
- Ichthyosis-hepatosplenomegaly-cerebellar degeneration syndrome
- Kufor-Rakeb syndrome
- Leukoencephalopathy-palmoplantar keratoderma syndrome
- MELAS
- Sporadic Creutzfeldt-Jakob disease
- Wolfram-like syndrome
- X-linked adrenoleukodystrophy
Common30–79%
27- ABeta amyloidosis, Italian type
- Adult-onset distal myopathy due to VCP mutation
- Atypical progressive supranuclear palsy syndrome
- Beta-propeller protein-associated neurodegeneration
- Bilateral striopallidodentate calcinosis
- Caribbean parkinsonism
- Congenital intrinsic factor deficiency
- Corticobasal syndrome
- Dentatorubral pallidoluysian atrophy
- Flynn-Aird syndrome
- Gaucher disease type 3
- Gerstmann-Straussler-Scheinker syndrome
- Huntington disease-like 1
- Ichthyosis follicularis-alopecia-photophobia syndrome
- Inherited Creutzfeldt-Jakob disease
- ITM2B amyloidosis
- Juvenile Huntington disease
- Lafora disease
- Metachromatic leukodystrophy, adult form
- NARP syndrome
- Neuronal intranuclear inclusion disease
- Pellagra
- Semantic dementia
- Sneddon syndrome
- Spinocerebellar ataxia type 2
- Subacute sclerosing leukoencephalitis
- X-linked spinocerebellar ataxia type 4
Sometimes5–29%
36- Adult Krabbe disease
- Adult-onset multiple mitochondrial DNA deletion syndrome due to DGUOK deficiency
- Adult polyglucosan body disease
- Autosomal dominant spastic paraplegia type 9A
- Autosomal dominant spastic paraplegia type 9B
- Autosomal recessive spastic paraplegia type 11
- Autosomal recessive spastic paraplegia type 46
- CADASIL
and 28 more in this range
The range is how often this sign appears among people diagnosed with each disease — it is not a probability of having any of them. Many of these signs also occur in common, non-rare conditions, and Orphanet separately records 5 diseases where this sign is specifically absent. A sign on this page is a conversation to have with a clinician, not a conclusion.
Part of the broader category
Also called: Progressive dementia
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Start free with EleplanThis page uses the Human Phenotype Ontology (2026-06-23) — Human Phenotype Ontology Consortium, hpo.jax.org. Disease associations from Orphanet (INSERM), CC BY 4.0. Content is educational and is not medical advice.