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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

  • 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
  • OMIM: 611637
  • UMLS: C0154682  C1968845
  • MeSH: -
  • GARD: -
  • MedDRA: 10036704
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