syndromic X-linked intellectual disability Hedera type

MeSH: C564516ORPHA: 93952

Overview

syndromic X-linked intellectual disability characterized by mild to moderate mental retardation and epilepsy that has material basis in mutation in the ATP6AP2 gene on chromosome Xp11

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with syndromic X-linked intellectual disability Hedera type, sourced from HPO and Orphanet clinical annotations.

Intellectual disabilityBilateral tonic-clonic seizureDelayed speech and language developmentMotor delayUnsteady gaitHyporeflexia of lower limbsExtrapyramidal muscular rigidityAtonic seizureHyporeflexia of upper limbsHypomimic faceCerebellar atrophyGait disturbanceDysmetriaSlurred speechObesityWeak voiceLeft ventricular hypertrophyPes planusCalcaneovalgus deformityHypoplasia of the corpus callosumApraxiaDroolingAction tremorFrequent fallsInability to walkScoliosisAbsent Achilles reflexBabinski signAstereognosisEcholaliaAgraphesthesia

Classification & Codes

MeSH Code

C564516

Orphanet Code

ORPHA:93952
syndromic X-linked intellectual disability Hedera type
MeSHC564516
OrphanetORPHA:93952
Treatments0 drug(s)
Symptoms on record31 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO