spinal muscular atrophy with progressive myoclonic epilepsy

MeSH: C537563ORPHA: 2590

Overview

rare neurodegenerative disease whose symptoms include slowly progressive muscle wasting

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 spinal muscular atrophy with progressive myoclonic epilepsy, sourced from HPO and Orphanet clinical annotations.

SeizureMyoclonusAbnormal lower motor neuron morphologyFunctional motor deficitLower limb muscle weaknessAbnormal circulating enzyme concentration or activityAtypical behaviorTremorRecurrent aspiration pneumoniaGeneralized myoclonic seizureClumsinessFrequent fallsRespiratory insufficiency due to muscle weaknessAtonic seizureTypical absence seizureSensorineural hearing impairmentMental deteriorationHigh-frequency sensorineural hearing impairmentWaddling gaitInability to walkScoliosisRespiratory failureEyelid myoclonusBilateral tonic-clonic seizure with generalized onsetMyoclonic status epilepticusLimb myoclonusIntellectual disabilityDysphagiaGait disturbance

Classification & Codes

MeSH Code

C537563

Orphanet Code

ORPHA:2590
spinal muscular atrophy with progressive myoclonic epilepsy
MeSHC537563
OrphanetORPHA:2590
Treatments0 drug(s)
Symptoms on record29 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
spinal muscular atrophy with progressive myoclonic epilepsy | OrphanDrug