Ataxia-telangiectasia-like disorder 1

ORPHA: 251347

Overview

human disease

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 Ataxia-telangiectasia-like disorder 1, sourced from HPO and Orphanet clinical annotations.

AtaxiaMask-like faciesSlow saccadic eye movementsOculomotor apraxiaDysarthriaCerebellar atrophyGeneralized hypotoniaDysmetriaReduced tendon reflexesCerebellar vermis hypoplasiaDystoniaGait ataxiaChoreaIntention tremorDilated fourth ventricleOrofacial dyskinesiaAbsent Achilles reflexEnlarged interhemispheric fissureAbnormality of ocular smooth pursuitGaze-evoked nystagmusDysmetric saccadesDelayed speech and language developmentHypergonadotropic hypogonadismMyoclonusJoint hypermobilityPes cavusDysdiadochokinesisDroolingFrequent fallsHyperactive deep tendon reflexesSensorimotor neuropathyVertical nystagmusSmall posterior fossaShort stature

Classification & Codes

Orphanet Code

ORPHA:251347
Ataxia-telangiectasia-like disorder 1
OrphanetORPHA:251347
Treatments0 drug(s)
Symptoms on record34 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO