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Rolandic epilepsy-speech dyspraxia syndrome

ORPHA:163721DiseaseAutosomal dominant, X-linked dominantAntenatal, Infancy, Neonatal

Ассоциированные гены (2)

SRPX2
sushi repeat containing protein X-linked 2
Disease-causing germline mutation(s) in
OMIM: 300642
GRIN2A
glutamate ionotropic receptor NMDA type subunit 2A
Disease-causing germline mutation(s) in
OMIM: 138253

Фенотипы (18)

Очень частый (80–99%)3
HP:0000750Delayed speech and language development
HP:0001250Seizure
HP:0011098Speech apraxia
Частый (30–79%)7
HP:0001249Intellectual disability
HP:0002307Drooling
HP:0007334Bilateral tonic-clonic seizure with focal onset
HP:0007359Focal-onset seizure
HP:0011196EEG with focal sharp waves
HP:0011198EEG with generalized epileptiform discharges
HP:0031491Continuous spike and waves during slow sleep
Периодический (5–29%)8
HP:0000736Short attention span
HP:0001260Dysarthria
HP:0001328Specific learning disability
HP:0001611Hypernasal speech
HP:0002079Hypoplasia of the corpus callosum
HP:0002546Incomprehensible speech
HP:0010300Abnormally low-pitched voice
HP:0031434Abnormal speech prosody

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы