rigid spine syndrome

MeSH: C535683ORPHA: 3246041 Treatment Available

Overview

human disease

Available Treatments (1)

DrugFormStatusCountriesLead Time
idebenone
Orphan
oral tablet, 150 mgEMA Approved921d

Clinical Presentation

Signs and symptoms associated with rigid spine syndrome, sourced from HPO and Orphanet clinical annotations.

Muscular dystrophyHigh palateGeneralized hypotoniaFailure to thriveAbnormally high-pitched voiceRestrictive ventilatory defectDelayed gross motor developmentPoor head controlScoliosisMultiple joint contracturesNocturnal hypoventilationSpinal rigidityAxial muscle weaknessGeneralized amyotrophyShort statureIntermittent episodes of respiratory insufficiency due to muscle weaknessLimited neck flexionIncreased muscle lipid contentWeakness of facial musculatureMuscle fiber atrophyMandibular prognathiaMicroretrognathiaHip dysplasiaMitral valve prolapseCongestive heart failureRight ventricular hypertrophyRight ventricular failurePes planus

Classification & Codes

MeSH Code

C535683

Orphanet Code

ORPHA:324604
rigid spine syndrome
MeSHC535683
OrphanetORPHA:324604
Treatments1 drug(s)
Symptoms on record28 signs
Statuspublished

Treatment Summary

Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO