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Progressive supranuclear palsy-progressive non-fluent aphasia syndrome

ORPHA:240112Clinical subtypeNot applicableAdult

Ассоциированные гены (1)

MAPT
microtubule associated protein tau
Major susceptibility factor in
OMIM: 157140

Фенотипы (21)

Очень частый (80–99%)7
HP:0011098Speech apraxia
HP:0006977Grammar-specific speech disorder
HP:0030391Spoken Word Recognition Deficit
HP:0002549Deficit in phonologic short-term memory
HP:0030784Anomia
HP:0025268Stuttering
HP:0002167Abnormality of speech or vocalization
Частый (30–79%)5
HP:0001268Mental deterioration
HP:0002465Poor speech
HP:0009088Speech articulation difficulties
HP:0031434Abnormal speech prosody
HP:0000741Apathy
Периодический (5–29%)9
HP:0007158Progressive extrapyramidal muscular rigidity
HP:0000511Vertical supranuclear gaze palsy
HP:0002527Falls
HP:0001300Parkinsonism
HP:0002312Clumsiness
HP:0002015Dysphagia
HP:0002172Postural instability
HP:0002381Aphasia
HP:0030217Limb apraxia

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы