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Primary lateral sclerosis

Disease definition

Primary lateral sclerosis (PLS) is an idiopathic non-familial motor neuron disease characterized by slowly progressive upper motor neuron dysfunction leading to spasticity, mild weakness in voluntary muscle movement, hyperreflexia, and loss of motor speech production.

ORPHA:35689

Classification level: Disorder
  • Synonym(s):
    • Adult-onset PLS
    • Adult-onset primary lateral sclerosis
    • PLS
  • Prevalence: 1-9 / 100 000
  • Inheritance: Not applicable or Autosomal recessive or Autosomal dominant 
  • Age of onset: Elderly, Adult
  • ICD-10: G12.2
  • ICD-11: 8B60.4
  • OMIM: 611637
  • UMLS: C0154682
  • MeSH: -
  • GARD: 10684
  • MedDRA: 10036704

Detailed information

Clinical Outcome Assessment (COA)

  • Patient-Centered Outcome Measures (PCOMs)
  • English (2023) - PROQOLIDTM
ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.