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Self-limited epilepsy with centrotemporal spikes

ORPHA:1945DiseaseAutosomal dominantChildhood

Ассоциированные гены (3)

GABRG2
gamma-aminobutyric acid type A receptor subunit gamma2
Disease-causing germline mutation(s) in
OMIM: 137164
GRIN2A
glutamate ionotropic receptor NMDA type subunit 2A
Disease-causing germline mutation(s) in
OMIM: 138253
SRPX2
sushi repeat containing protein X-linked 2
Candidate gene tested in
OMIM: 300642

Фенотипы (21)

Очень частый (80–99%)1
HP:0012557EEG with centrotemporal focal spike waves
Частый (30–79%)7
HP:0002307Drooling
HP:0007332Focal hemifacial clonic seizure
HP:0007334Bilateral tonic-clonic seizure with focal onset
HP:0007359Focal-onset seizure
HP:0009088Speech articulation difficulties
HP:0010535Sleep apnea
HP:0025425Laryngospasm
Периодический (5–29%)11
HP:0000712Emotional lability
HP:0000716Depression
HP:0000736Short attention span
HP:0000739Anxiety
HP:0001328Specific learning disability
HP:0002076Migraine
HP:0002373Febrile seizure (within the age range of 3 months to 6 years)
HP:0003401Paresthesia
HP:0006889Intellectual disability, borderline
HP:0007018Attention deficit hyperactivity disorder
HP:0012534Dysesthesia
Очень редкий (1–4%)2
HP:0001326EEG with irregular generalized spike and wave complexes
HP:0007270Atypical absence seizure

Эпидемиология (2)

Point prevalence
Unknown
Worldwide
Annual incidence
1-5 / 10 000
Sweden

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы