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1 OMIM reference -
6 associated genes
No signs/symptoms info
PROTEIN INTERACTIONS: 1
1 OMIM reference -
1 associated gene
14 signs/symptoms
Progressive non-fluent aphasia
Perry syndrome

C9ORF72 DCTN1
CHMP2B
GRN
MAPT
PSEN1
VCP


INTERACTOME
ASSOCIATIONS

(click on a score value to see the evidence)
MAPT
(0.75)
DCTN1



Citations in the biomedical literature:


Progressive non-fluent aphasia
C9ORF72 CHMP2B GRN MAPT PSEN1 VCP

Perry syndrome
DCTN1



Progressive non-fluent aphasia
Perry syndrome

Synonym(s):
- Agramatic variant of PPA
- Agramatic variant of primary progressive aphasia
- Non-fluent variant PPA

Synonym(s):
- Parkinsonism with alveolar hypoventilation and mental depression

Classification (Orphanet):
- Rare genetic disease
- Rare neurologic disease
Classification (Orphanet):
- Rare genetic disease
- Rare neurologic disease

Classification (ICD10):
- Diseases of the nervous system -
Classification (ICD10):
(no data available)

Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: adulthood
Average age of death: -
Type of inheritance: multigenic/multifactorial
Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: adulthood
Average age of death: -
Type of inheritance: autosomal dominant

External references:
1 OMIM reference -
1 MeSH reference: D057178
External references:
1 OMIM reference -
No MeSH references

Perry syndrome

Very frequent
- Autosomal dominant inheritance
- Execution movement disorder / dysmetria / bradykinesia / akinesia / apraxia
- Extrapyramidal syndrome
- Humour troubles / anxiety / depression / apathy / euphoria / irritability
- Respiratory distress / dyspnea / respiratory failure / lung volume reduction
- Sleep and vigilance disorders
- Tremor
- Weight loss / loss of appetite / break in weight curve / general health alteration

Occasional
- Delirium / hallucination
- Early death / lethality
- Hypotension
- Psychic / behavioural troubles
- Psychic / psychomotor regression / dementia / intellectual decline
- Psychosis / schizophrenia / maniac disorder


Progressive non-fluent aphasia

(no data available)