Ullrich congenital muscular dystrophy

ORPHA: 75840

Overview

Ullrich congenital muscular dystrophy (UCMD) is characterized by early-onset, generalized and slowly progressive muscle weakness, multiple proximal joint contractures, marked hypermobility of the distal joints and normal intelligence

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 Ullrich congenital muscular dystrophy, sourced from HPO and Orphanet clinical annotations.

Abnormal palate morphologyGeneralized hypotoniaFlexion contractureKyphosisElevated circulating creatine kinase concentrationSpinal rigidityGeneralized muscle weaknessEMG: myopathic abnormalitiesIncreased variability in muscle fiber diameterAbnormal muscle fiber morphologyWrist hypermobilityIncreased laxity of fingersIncreased endomysial connective tissueMicrognathiaShort neckTorticollisEsotropiaAdducted thumbSlender fingerMuscle weaknessDecreased fetal movementFrequent fallsScoliosisHip dislocationRespiratory failureElbow flexion contractureGeneralized amyotrophyKnee flexion contracturePes valgusDiaphragmatic weaknessLong toe

Classification & Codes

Orphanet Code

ORPHA:75840
Ullrich congenital muscular dystrophy
OrphanetORPHA:75840
Treatments0 drug(s)
Symptoms on record31 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO