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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

  A summary on this disease is available in Español (2015) Italiano (2015) Nederlands (2015) Deutsch (2004) Français (2004) Português (2004)

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)

Additional information

Further information on this disease

Patient-centred resources for this disease

Research activities on this disease

Specialised Social Services

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.