progressive supranuclear palsy-progressive non-fluent aphasia syndrome
Findings
No curated finding names progressive supranuclear palsy-progressive non-fluent aphasia syndrome yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
PSP-progressive non fluent aphasia (PSP-PNFA) is an atypical variant of progressive supranuclear palsy (PSP), a rare late-onset neurodegenerative disease. Unlike classic PSP (Richardson syndrome) patients present with an isolated speech production problem years before developing other motor features of PSP.
Definition from the Mondo Disease Ontology (MONDO:0016564), read 2026-09-29. CC BY 4.0.
Features
21 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal speech patternHPOHP:0002167
- Very frequent (80% to 99% of cases)
- Anomic aphasiaHPOHP:0030784
- Very frequent (80% to 99% of cases)
- Deficit in grammarHPOHP:0006977
- Very frequent (80% to 99% of cases)
- Deficit in phonologic short-term memoryHPOHP:0002549
- Very frequent (80% to 99% of cases)
- Speech apraxiaHPOHP:0011098
- Very frequent (80% to 99% of cases)
- Spoken word recognition deficitHPOHP:0030391
- Very frequent (80% to 99% of cases)
Show the remaining 9
- AphasiaHPOHP:0002381
- Occasional (5% to 29% of cases)
- ClumsinessHPOHP:0002312
- Occasional (5% to 29% of cases)
- DysphagiaHPOHP:0002015
- Occasional (5% to 29% of cases)
- FallsHPOHP:0002527
- Occasional (5% to 29% of cases)
- Limb apraxiaHPOHP:0030217
- Occasional (5% to 29% of cases)
- ParkinsonismHPOHP:0001300
- Occasional (5% to 29% of cases)
Where it sits
Other names
3 names
Resolves to: progressive supranuclear palsy-progressive non-fluent aphasia syndrome
- Also called
- progressive supranuclear palsy-apraxia of speech syndromePSP-AOSPSP-PNFA